Hearing Loss & Aural Atresia

Hearing Loss and Microtia — What It Is, What It Isn't, and What It Means for Your Child

Every child born with microtia has some degree of hearing loss on the affected side. Understanding why — and what can be done — is an important part of navigating your child’s care. The vast majority of children with microtia have a completely normal inner ear and hearing nerve. What they have is a mechanical problem — sound cannot reach a working inner ear because the pathway doesn’t exist. That is very different from deafness, and it is treatable.

🔔
The key fact: In microtia, the inner ear and hearing nerve form at a different time than the outer and middle ear. In almost all cases, they develop normally. Your child’s hearing system works. The problem is access — not function.
Dr. Arturo Bonilla MD
Dr. Arturo Bonilla, MD — Written & Medically Reviewed
Fellowship-Trained · Pediatric Microtia Surgeon · Pediatric Otolaryngologist · Exclusively microtia since 1996 · Last reviewed 2026 · Updated regularly
✓ Medically Reviewed
The Key Distinction

Conductive Hearing Loss vs. Sensorineural Hearing Loss

These two terms describe fundamentally different problems — and confusing them is one of the most common sources of unnecessary anxiety for microtia families. Understanding the difference helps clarify what kind of hearing challenge your child actually has — and how addressable it is.

Conductive hearing loss

Conductive hearing loss means that sound cannot be conducted through the outer or middle ear to reach the inner ear. The inner ear itself — where hearing actually happens — is normal. The problem is mechanical and structural: the pathway for sound is blocked, absent, or malformed.

Think of it this way: imagine a fully functional speaker with a disconnected wire. The speaker works perfectly. The signal generator works perfectly. The problem is the connection between them. Conductive hearing loss is a connection problem — not a processing problem.

Children with microtia and aural atresia have conductive hearing loss. The inner ear works. The hearing nerve works. The pathway from outside world to inner ear is incomplete — but that pathway can be bypassed, reconstructed, or supplemented. This type of hearing loss is highly addressable.

Sensorineural hearing loss

Sensorineural hearing loss means the inner ear (cochlea) or the hearing nerve itself is damaged or absent. This is what most people picture when they think of deafness. It is a fundamentally different condition — harder to treat, not correctable by reconstructing the outer or middle ear.

In microtia, sensorineural hearing loss is rare. The inner ear develops on a different timeline than the outer ear — it is largely formed before the developmental disruption that causes microtia occurs. In the overwhelming majority of microtia cases, sensorineural hearing is completely normal.

The reassuring summary: Your child almost certainly has conductive hearing loss — not sensorineural loss. Their hearing system works. The problem is mechanical, structural, and addressable through hearing devices, surgical intervention, or both. They are not deaf.

What microtia causes
Conductive Hearing Loss
Sound cannot pass through the outer or middle ear to reach the inner ear. The inner ear itself is normal.
Inner ear (cochlea) is normal
Hearing nerve is normal
Sound can bypass the missing canal via bone conduction
Highly addressable — devices & surgery both work
Outer or middle ear structure absent or malformed
▶ This is what microtia causes
What microtia does NOT typically cause
Sensorineural Hearing Loss
The inner ear or hearing nerve itself is damaged or absent. The outer ear structure may be normal.
Cochlea or hearing nerve damaged
Cannot be corrected by outer/middle ear reconstruction
More complex to address — cochlear implants may be needed
Inner ear forms on a different developmental timeline — usually unaffected in microtia
Understanding the Anatomy

Why Microtia Causes Hearing Loss — The Developmental Explanation

The ear has three distinct anatomical regions that develop at different times and serve different functions. Understanding this is the key to understanding why microtia produces the specific kind of hearing loss it does — and why that is actually reassuring.

👂
Outer Ear
Auricle & Ear Canal
The visible external ear (auricle/pinna) and the ear canal (external auditory meatus) that carries sound to the eardrum. Collects and funnels sound waves toward the middle ear. Develops during the first trimester — the same period when the developmental disruption causing microtia occurs.
⚠ Affected in microtia
🥁
Middle Ear
Eardrum & Ossicles
The eardrum (tympanic membrane) and three tiny bones — malleus, incus, and stapes — that amplify and transmit vibration from the eardrum to the inner ear. Also develops during the first trimester. In microtia, the ossicles are often fused or malformed, and the middle ear cavity may be smaller than normal. Ear canal surgery can address this.
~ Often affected in microtia
🎶
Inner Ear
Cochlea & Hearing Nerve
The cochlea converts vibration into electrical signals that the hearing nerve carries to the brain. The vestibular system handles balance. The inner ear develops earlier — during the fourth to seventh weeks — before the developmental disruption that causes microtia. In the vast majority of microtia cases, the inner ear and hearing nerve are completely normal and fully functional.
✓ Usually completely normal

The developmental timing explanation: Microtia occurs because of a disruption to the blood supply or development of the outer ear structures during the first trimester. The inner ear forms earlier — it is largely complete before this disruption happens. This is why microtia almost always coexists with a normal inner ear. The outer and middle ear are affected; the inner ear is protected by its earlier developmental schedule.

Video Explanation

Watch Dr. Bonilla explain how the ear processes sound and how microtia affects hearing.

Term Explained
Aural Atresia
aural = relating to the ear  |  atresia = absence or closure of a body passage

Aural atresia specifically means the absence or abnormal closure of the ear canal (external auditory meatus). It is important to distinguish this from other medical uses of the word "atresia" — in other contexts, atresia refers to closure of different body passages (intestinal atresia, biliary atresia, etc.). When we say aural atresia, we are referring specifically and exclusively to the ear canal.

Most patients born with microtia also have aural atresia — the ear canal is absent or severely underdeveloped. Without an ear canal, sound waves cannot travel from the outside world to the eardrum and middle ear in the normal way.

The term you will encounter most frequently in clinical settings and surgical discussions is microtia with aural atresia — these two conditions almost always occur together.

Aural Atresia Explained

What Is Aural Atresia — and Why Does It Occur With Microtia?

Aural atresia is the medical term for the absence or abnormal closure of the ear canal. The word aural specifically indicates the ear — distinguishing this condition from other medical uses of "atresia" which describe closure of different body passages entirely.

Because the outer ear (auricle) and the ear canal develop from the same embryonic tissue at approximately the same time, a developmental disruption that causes one to form abnormally almost always affects the other. This is why microtia and aural atresia occur together in the vast majority of cases — they are two expressions of the same underlying developmental event. Families who want a broader understanding of microtia, its causes, and treatment options can find a full overview on the microtia page.

What aural atresia looks like anatomically

In a typical microtia patient with aural atresia, the ear canal is either completely absent — a solid plate of bone where the canal opening would be — or severely narrowed to a degree that provides no meaningful sound conduction. The middle ear is often small, and the ossicles (the three tiny sound-conducting bones) may be fused to each other or to the walls of the middle ear cavity.

The eardrum (tympanic membrane), if present at all, may be abnormally positioned or formed. A high-resolution CT scan of the temporal bone is used to map this anatomy precisely before any surgical intervention on the ear canal is considered.

Aural atresia and the facial nerve

One anatomical complexity worth understanding: the facial nerve — the nerve that controls all movements of the face — runs through the temporal bone very close to the structures affected by aural atresia. In some microtia patients, the facial nerve takes an atypical course through this region. This is one of the reasons CT imaging is performed before any ear canal surgery — to map the facial nerve’s exact position and assess surgical risk. In experienced hands, the risk of facial nerve injury during atresiaplasty is low, but it must be assessed individually for every patient.

A terminology note: You may also encounter the terms atresia auris, congenital aural atresia, or simply ear canal atresia — these all refer to the same condition. When reading medical literature or speaking with other specialists, aural atresia specifically and always refers to the ear canal, not any other structure.

Presentation & Urgency

Unilateral vs. Bilateral Microtia — Why the Distinction Matters for Hearing

Whether microtia affects one ear or both ears has profound implications for hearing urgency, intervention timing, and the consequences of delay. This is the most important variable in hearing management.

One side affected
Unilateral Microtia
(& Aural Atresia)
The other ear is typically completely normal — providing bilateral hearing input
Children with one normal ear do not miss developmental language milestones — the normal ear provides sufficient input
Sound localization (determining where sound comes from) is affected — this is worth monitoring
Hearing intervention is beneficial but not an emergency — waiting is a reasonable option with monitoring
Bone-anchored hearing solutions can improve hearing symmetry and classroom hearing
The decision to intervene can be made with time for thoughtful evaluation
Both sides affected
Bilateral Microtia
(& Aural Atresia)
Both ears have conductive hearing loss — no ear is providing normal sound input
Language development, speech acquisition, and learning are at risk without intervention
Intervention is not optional — bone-anchored hearing devices must be provided as early as possible, from infancy
A pediatric audiologist must be involved from the first weeks of life
BAHA soft-band devices can be used in infants from approximately 3–4 months of age
Children who receive early bone conduction amplification develop language normally
⚠ Urgent — begin from birth
>95%
Of microtia patients have a completely normal inner ear
~90%
Of microtia cases are unilateral — one ear affected
~10%
Are bilateral — both ears affected, requiring immediate hearing support
3mo
Minimum age for soft-band bone-anchored hearing device fitting
Hearing by Age

A Developmental Timeline for Hearing Management

Hearing management in microtia is not a single decision — it is a sequence of evaluations and interventions that evolves as the child grows. Here is what to expect at each stage.

The most important thing to know: for unilateral cases, there is room for thoughtful decision-making. For bilateral cases, hearing support from infancy is essential and non-negotiable. Both paths lead to children who hear well and develop normally.

Hearing management and ear reconstruction are addressed separately — on separate timelines with separate specialists. They can be coordinated, but neither is a prerequisite for the other. Dr. Bonilla addresses both in his practice and can discuss the full picture at consultation.

Newborn hearing screening: Most hospitals screen newborns for hearing at birth using OAE (otoacoustic emissions) or ABR (auditory brainstem response) testing. A child with unilateral microtia may pass the screening in the normal ear and fail or be referred on the affected side — this is expected and is not a cause for alarm. Follow the recommendation for further audiological evaluation.

0
Birth — First weeks
Newborn Hearing Screening & Audiological Referral
Bilateral: Engage a pediatric audiologist immediately. Bone conduction hearing support should be planned from the earliest weeks. Unilateral: Follow up on the newborn hearing screen. Begin audiological monitoring. Normal development expected with one hearing ear.
3m
3–6 Months
Soft-Band BAHA / Bone-Conduction Device
Non-surgical bone-anchored hearing devices (worn on a soft elastic headband) can be fitted as early as 3–4 months. For bilateral cases this is essential. For unilateral cases it is optional but beneficial — improves sound localization and provides a fuller hearing experience.
2–4
Ages 2–4
Monitoring & Audiological Assessment
Regular audiological follow-up to monitor hearing function and language development. Children with bilateral microtia wearing bone-conduction devices typically meet all language milestones. No surgical hearing intervention yet — still too young.
5+
Ages 5–6
CT Scan & Surgical Candidacy Assessment
High-resolution CT scanning of the temporal bone is performed to assess anatomy for atresiaplasty candidacy: middle ear and mastoid pneumatization, ossicle anatomy, inner ear morphology, and facial nerve course. This determines whether surgical ear canal reconstruction is feasible and advisable.
6–9
Ages 6–9
Ear Reconstruction & Hearing Surgery Planning
Ear reconstruction with rib cartilage typically begins at age 6. Atresiaplasty (ear canal surgery) is typically planned separately, often after ear reconstruction is underway or complete — the sequencing depends on the individual anatomy and is discussed with the surgical team. Surgical bone-anchored hearing implants can also be considered at this age.
Long-term
Completed Reconstruction — Permanent Result
After ear reconstruction and hearing intervention are complete, most patients have a permanent, natural-looking ear and either a surgically created hearing pathway (atresiaplasty) or a bone-anchored hearing solution. Children who navigated this process with good audiological support develop normal speech, language, and academic outcomes.
For Parents

A Parent's Guide to Understanding Your Child's Hearing Test

When your child is diagnosed with microtia, one of the first things you will encounter is a hearing test — and the results can feel overwhelming if you have never seen an audiogram before. Dr. Bonilla walks you through exactly what the numbers mean, what normal hearing looks like on a graph, and what conductive hearing loss — the type almost always associated with microtia — looks like. Understanding this single document will help you ask better questions and make more confident decisions about your child's hearing care.

Dr. Bonilla explains how to read your child's audiogram and what the results mean for hearing intervention.

👈
What is an Audiogram

An audiogram is a graph that maps your child's hearing ability across different frequencies and volumes. It is the roadmap for all hearing decisions.

📊
What Normal Hearing Looks Like

Normal hearing registers between 0 and 25 decibels across all frequencies. Most microtia patients show normal inner ear function at these levels when tested by bone conduction.

🔊
Conductive Hearing Loss

Microtia with aural atresia causes conductive hearing loss — meaning sound cannot reach the inner ear through the ear canal. The inner ear itself is almost always completely normal and working perfectly.

What the Results Mean

A normal bone conduction result on the audiogram is genuinely good news — it confirms the cochlea and hearing nerve are intact and that hearing intervention will be highly effective for your child.

Hearing Treatment Options

Four Approaches to Hearing in Microtia — and When Each Is Appropriate

Hearing surgery is not mandatory for all microtia patients. The right approach depends on whether microtia is unilateral or bilateral, the anatomy of the ear canal and middle ear, and the family’s priorities and circumstances.

First-line for all ages
📊
Non-Surgical Bone-Conduction Hearing Device
Soft-band BAHA (Cochlear/Oticon) / Adhear
A bone-conducting hearing processor worn on a soft elastic headband (or adhered to the skin) transmits sound directly through the skull to the inner ear — bypassing the absent ear canal entirely. This is the safest, most immediate hearing solution for all ages, including infants as young as 3–4 months. For bilateral microtia, this is the essential first intervention and should begin from infancy.
Best for: All ages, including infants. Bilateral cases urgently. Unilateral cases optionally.
Surgical? No — worn on headband or skin adhesive.
Key brands: Cochlear BAHA Softband, Oticon Ponto, Med-El Adhear
Surgical option
🧬
Surgical Bone-Anchored Hearing Implant
Cochlear Osia / Oticon Sentio / MED-EL Bonebridge
A titanium implant is placed in the skull behind the ear. A sound processor attaches to this implant (either magnetically, or via an abutment that protrudes through the skin) and transmits sound through bone conduction to the inner ear. This provides a more stable, powerful bone-conduction hearing solution than a headband device — and is the long-term option for patients who want bone-anchored hearing without undergoing atresiaplasty. Dr. Bonilla performs osseointegrated BAHA implant surgery concurrently with ear reconstruction where appropriate, eliminating the need for a separate surgical procedure and an additional episode of general anesthesia.
Best for: Age 5+ who are not atresiaplasty candidates or who prefer a permanent bone-anchored solution.
Surgical? Yes — titanium implant in skull.
System types: Osia (Cochlear) · Sentio (Oticon) · Bonebridge (MED-EL) — all subcutaneous, no visible abutment
Surgical — hearing canal reconstruction
🚨
Atresiaplasty
Ear Canal & Eardrum Reconstruction Surgery
Atresiaplasty is a specialized surgical procedure in which a specialized drill creates an ear canal through the bone behind the existing microtia, a new eardrum is created, and a skin graft lines the new canal. When successful, this restores conventional sound conduction — the child hears through a reconstructed pathway. Not all patients are candidates — the anatomy of the middle ear, ossicles, facial nerve course, and mastoid pneumatization must be assessed by CT scanning first. This procedure requires the expertise of an experienced otologist or pediatric otolaryngologist.
Best for: Patients with favorable CT anatomy — good mastoid, accessible ossicles, safe facial nerve course.
Surgical? Yes — complex, specialized procedure.
Primary risk: Canal re-stenosis; facial nerve injury (low risk in experienced hands)
Unilateral cases — a valid choice
Active Monitoring (No Intervention)
Observation with Audiological Follow-up
For children with unilateral microtia and aural atresia, choosing not to pursue hearing intervention is a legitimate option — provided the child is monitored. One fully hearing ear is sufficient for normal language development, academic performance, and quality of life in the majority of cases. The decision to monitor rather than intervene should be made with full information from the audiological team, and should be revisited as the child grows and can express their own preferences.
Appropriate for: Unilateral microtia only. Not appropriate for bilateral cases.
Requirement: Regular audiological monitoring; re-evaluation if learning concerns arise.
Note: This decision can be revised at any time — choosing to wait does not foreclose future options.
Bone-Conduction Hearing Systems

Bone-Conduction Hearing Devices for Microtia Patients

These are the principal non-surgical and surgical bone-conduction systems used for microtia patients. Your audiologist will recommend the most appropriate option based on your child’s age, anatomy, and hearing levels.

Cochlear Americas
Baha System/OSIA®
Non-surgical soft-band & surgical implant options
The Baha system offers both soft-band (headband) and surgical implant options. The surgical Baha Osia uses subcutaneous magnets — no visible abutment. Soft-band versions available for infants from ~3 months. Widely available and extensively studied.
Ponto® System/Sentio
Ponto® System/Sentio
Soft-band & surgical subcutaneous magnet options
The Ponto system is a well-regarded alternative to the Baha system. Offers soft-band and surgical subcutaneous magnet-based options. Known for high-quality sound processing and good feedback management.
Med-El
Adhear
Skin adhesive (non-surgical) option
The Adhear is a newer bandless option — the processor adheres directly to an adapter stuck behind the ear, eliminating the headband.

Device selection guidance: The "best" device is the one that fits your child’s age, anatomy, hearing levels, and lifestyle. An experienced pediatric audiologist is the right professional to guide this choice. Dr. Bonilla’s team can provide referrals to audiologists experienced with microtia patients if needed.

How Reconstruction Relates to Hearing

Ear Reconstruction and Hearing Restoration — Two Separate Treatment Decisions

One of the most common misconceptions families have is that rebuilding the external ear will automatically restore hearing. It is essential to clarify this: external ear reconstruction and hearing restoration are separate procedures addressing separate anatomical problems.

Reconstructing the visible external ear (the auricle) with rib cartilage restores the appearance of the ear and provides a permanent, biological structure. It does not, by itself, create an ear canal or restore conventional sound conduction. Hearing is addressed through a separate pathway — either atresiaplasty, a bone-anchored hearing implant, or continued use of non-surgical bone-conduction devices.

Ear Reconstruction
Rebuilds the visible external ear structure using natural rib cartilage. Performed in 1 to 3 stages beginning around age 6–9 depending on grade. Addresses appearance — does not create an ear canal.
Hearing Restoration
Addresses the hearing loss through bone-conduction devices, surgical bone-anchored implants, or atresiaplasty (canal reconstruction). Planned and performed independently of ear reconstruction.
Sequencing
In most cases, ear reconstruction is performed first, followed by hearing surgery. This sequencing protects the tissue needed for reconstruction. Dr. Bonilla discusses the specific plan for each child at consultation.
The Permanent Result
After reconstruction is complete, patients have a permanent, biological external ear — and a hearing solution that is either permanent (bone-anchored implant, atresiaplasty) or ongoing (non-surgical device). Most lead full, unrestricted lives with excellent hearing outcomes.

Dr. Bonilla coordinates with audiologists, otologists, and atresiaplasty specialists to ensure the full care plan is coherent and appropriately sequenced for each child.

Common Questions

Questions Families Ask About Hearing and Microtia

Will my child with unilateral microtia develop normal speech and language?
+

In the overwhelming majority of cases, yes. Children with one fully hearing ear develop normal speech and language — the normal ear provides sufficient auditory input for all developmental milestones. Language delays are not typically associated with unilateral conductive hearing loss when the other ear is normal.

The areas that may be affected are sound localization (determining the direction of sounds) and hearing in noisy environments — a school cafeteria or a loud classroom can be more challenging for a child who is listening with one ear. These are real considerations worth discussing with an audiologist, but they are manageable and should not alarm parents.

Will a bone-anchored hearing device help my child hear like a normal ear?
+

Bone-anchored hearing devices are highly effective. For unilateral microtia patients, they deliver sound from the affected side to the working inner ear — providing a more complete binaural hearing experience. For bilateral microtia patients, they are the primary mechanism for hearing, and when appropriately fitted, allow children to reach normal language milestones.

They do not replicate the experience of a normal ear with a full ear canal — the sound quality, while good, is different from conventional hearing. But they provide meaningful, functional hearing that makes a substantial difference in learning environments and daily life. Many children adapt to them quickly and find them helpful enough to wear consistently.

Is atresiaplasty always recommended? What if my child’s anatomy doesn’t support it?
+

Atresiaplasty is not appropriate for every patient — in fact, a meaningful number of microtia patients do not have anatomy that supports it safely. The CT scan assessment of the temporal bone determines candidacy. Key factors that make a patient a good candidate include: well-pneumatized mastoid, reasonably normal ossicle anatomy, favorable facial nerve course, and adequate middle ear space.

If the CT scan shows poor anatomy for atresiaplasty — an underdeveloped mastoid, severely malformed ossicles, or an anomalous facial nerve course that increases surgical risk — the otologist will recommend against it. In this case, bone-anchored hearing solutions (non-surgical or surgical) remain fully effective and are the appropriate long-term hearing management. Not being an atresiaplasty candidate does not mean living with poor hearing — it means choosing the appropriate hearing solution for that anatomy.

How does a bone-anchored hearing device differ from a conventional hearing aid?
+

A conventional hearing aid amplifies sound and delivers it through the ear canal. This requires a functioning ear canal — which children with microtia and aural atresia do not have. Conventional hearing aids simply do not work for aural atresia patients, because there is no canal to deliver the amplified sound through.

A bone-anchored hearing device bypasses the ear canal entirely. Instead of delivering sound through air in a canal, it transmits vibrations through the skull bone directly to the cochlea. Because the cochlea in microtia patients is usually completely normal, this is highly effective — the device delivers sound directly to a fully working inner ear, simply via a different physical pathway.

We’ve been told to wait on any hearing intervention. Should we?
+

The answer depends entirely on whether microtia is unilateral or bilateral.

For unilateral microtia: Waiting and monitoring is a legitimate and defensible approach, provided audiological evaluation is performed and development is tracked. The normal ear compensates effectively in most situations. Choosing to wait does not harm the child's development in most cases.

For bilateral microtia: Waiting on hearing intervention is not appropriate. Both ears have conductive hearing loss, and without auditory input, language development is at significant risk. If you have been told to wait on a child with bilateral microtia and no hearing device has been provided, please seek a second opinion from a pediatric audiologist with microtia experience immediately.

Does Dr. Bonilla perform atresiaplasty?
+

No. Dr. Bonilla refers patients who are eligible candidates for atresiaplasty to neurotologist specialists for further evaluation and surgical management. He coordinates the sequencing of outer ear reconstruction with the referring neurotologist to ensure the timing of canal surgery does not compromise the reconstruction. For patients who are not atresiaplasty candidates or who elect not to pursue canal reconstruction, Dr. Bonilla performs bone-anchored hearing implant surgery concurrently with ear reconstruction — eliminating a separate surgical procedure and additional anesthesia. Families do not need to navigate these decisions independently — the full care plan is coordinated through Dr. Bonilla’s team from the initial consultation.

Will my child need to wear a hearing device for life, even after ear reconstruction?
+

That depends on whether atresiaplasty is performed and successful. If ear canal reconstruction (atresiaplasty) is performed and achieves a functioning hearing pathway, the child may be able to hear through that reconstructed canal without a device — or with only a conventional hearing aid if the result is partial.

If atresiaplasty is not performed — either because the anatomy is not suitable or the family chooses not to pursue it — the bone-anchored hearing solution (non-surgical or surgical implant) becomes the long-term hearing approach. Surgical bone-anchored implants are a permanent solution that functions reliably without ongoing device management beyond processor care and battery replacement.

Ear reconstruction (building the external ear from cartilage) does not, by itself, restore hearing — it restores appearance. The hearing question is addressed separately, through the pathways described on this page.

Contact Dr. Bonilla’s Office

Dr. Bonilla’s team coordinates the full care picture for microtia families — ear reconstruction, hearing management, and atresiaplasty candidacy. To get started, submit your information through our contact form and his team will be in touch to schedule a virtual consultation.