Multidisciplinary Management of Microtia in Infants
An infant diagnosed with microtia requires immediate audiologic assessment, early hearing intervention with bone-anchored hearing aids or conventional amplification by 6 months of age if hearing loss is confirmed, high-resolution CT of the temporal bone to assess candidacy for surgical interventions, and coordinated multidisciplinary care involving otolaryngology, audiology, plastic surgery, and speech-language pathology, with autologous rib-cartilage reconstruction typically deferred until age 6-10 years. 1, 2, 3, 4
Immediate Diagnostic Evaluation
Audiologic Assessment Timeline
Perform comprehensive audiologic evaluation by 3 months of age to confirm the presence and degree of conductive hearing loss, which is nearly universal in microtia with aural atresia. 5, 3, 4
Use auditory brainstem response (ABR) testing as the primary diagnostic modality in infants under 6 months, as behavioral audiometry is not yet reliable at this age. 5
Include otoacoustic emissions (OAE) and tympanometry in the initial assessment battery to evaluate cochlear function and middle ear status. 5
Obtain bone-conduction thresholds to differentiate conductive from sensorineural components, as approximately 10-15% of microtia cases have associated sensorineural hearing loss. 3, 4
Imaging Studies
Obtain high-resolution CT of the temporal bone between 3-6 months of age to evaluate middle ear anatomy, ossicular chain development, facial nerve course, and cochlear structure—all critical for determining candidacy for atresiaplasty or bone-anchored hearing aid placement. 5, 3, 4
Do not delay CT imaging beyond 6 months if surgical hearing intervention is being considered, as early anatomic assessment guides the timing and type of hearing rehabilitation. 3, 4
MRI is not routinely indicated unless there is suspicion of cochlear nerve abnormality based on ABR findings suggesting auditory neuropathy. 5
Hearing Rehabilitation Strategy
Amplification Timing and Selection
Fit amplification devices within 1 month of confirmed hearing loss diagnosis, ideally by 6 months of age, to prevent speech and language delays during critical developmental periods. 5
For unilateral microtia with normal contralateral hearing, consider bone-conduction hearing aids or BAHA on a softband starting at 3-6 months of age, though evidence for benefit in unilateral loss is mixed and family preference should guide this decision. 5, 3, 4
For bilateral microtia, bilateral bone-conduction amplification is mandatory by 6 months of age to provide access to speech sounds and prevent severe language delay. 5, 3, 4
Use bone-conduction devices on softbands initially, as surgical implantation of bone-anchored hearing aids requires adequate skull thickness, typically achieved by age 5 years. 3, 4
Surgical Hearing Intervention Timeline
Surgical bone-anchored hearing aid (BAHA) implantation can be performed at age 5 years or older once skull bone thickness is adequate (typically ≥3-4 mm) to support osseointegration. 3, 4
Atresiaplasty (surgical creation of an ear canal) should be considered only in carefully selected cases with favorable anatomy on CT (Jahrsdoerfer score ≥7), typically performed at age 5-6 years if pursued. 3, 4
Atresiaplasty has a 50-60% long-term success rate for achieving serviceable hearing and carries risks of facial nerve injury, restenosis, and cholesteatoma formation—making BAHA the preferred option for most patients. 3, 4
Auricular Reconstruction Planning
Timing of Reconstruction
Autologous rib-cartilage reconstruction should be performed at age 6-10 years, when the chest wall has adequate cartilage volume and the contralateral ear has reached approximately 85% of adult size (typically by age 6). 1, 2, 3, 4, 6
Two-stage autologous reconstruction is the gold standard, with stage 1 involving cartilage framework fabrication and implantation, and stage 2 (6-12 months later) involving elevation of the reconstructed ear and creation of the postauricular sulcus. 1, 6
Prosthetic ear placement is an alternative for patients who decline surgery, prefer to avoid multiple operations, or have medical contraindications to autologous reconstruction, and can be fitted at any age after 3-4 years. 1, 2, 4
Coordination with Hearing Surgery
If atresiaplasty is planned, perform it 6-12 months before auricular reconstruction to avoid compromising blood supply to the reconstructed ear framework. 3, 4
If BAHA implantation is planned, it can be performed before, during, or after auricular reconstruction, though many surgeons prefer to place the BAHA abutment during the first stage of ear reconstruction to minimize the total number of surgeries. 3, 4
Close communication between the otolaryngologist and plastic surgeon is essential to coordinate the timing and sequence of all interventions. 3, 4
Speech-Language Therapy
Early Intervention Enrollment
Refer to early intervention services within 2 days of confirmed hearing loss diagnosis, as mandated by federal EHDI guidelines, to support language development during the critical first 3 years of life. 5
Children with bilateral microtia and hearing loss who receive amplification and early intervention by 6 months of age achieve language development within the normal range by age 5 years, whereas those identified later have significantly poorer outcomes. 5
Ongoing Speech-Language Assessment
Perform speech-language evaluations at 6-month intervals from age 6-18 months, then annually through school age, to monitor vocabulary, receptive and expressive language, syntax, and speech production. 5, 7, 8
Children with unilateral microtia may have difficulty with sound localization and speech perception in noise, warranting speech-language assessment even when hearing thresholds in the normal ear are adequate. 5
Multidisciplinary Team Composition
Core Team Members
Pediatric otolaryngologist for diagnosis, hearing management, and surgical planning for atresiaplasty or BAHA. 1, 2, 3, 4
Audiologist for hearing assessment, amplification fitting, and long-term monitoring. 5, 1, 3, 4
Pediatric plastic surgeon for auricular reconstruction planning and execution. 1, 2, 3, 4
Speech-language pathologist for language assessment and therapy. 5, 1, 3, 4
Clinical geneticist for evaluation of syndromic associations, as 30-40% of microtia cases are associated with syndromes such as Goldenhar, Treacher Collins, or hemifacial microsomia. 5, 7, 8
Ophthalmologist for vision screening, as ocular abnormalities occur in 10-15% of microtia cases. 5
Additional Specialists as Needed
Pediatric cardiologist if syndromic features suggest cardiac involvement (e.g., CHARGE syndrome, 22q11.2 deletion). 7, 8
Pediatric nephrologist if renal anomalies are suspected, as branchio-oto-renal syndrome can present with microtia. 5
Orthodontist for management of jaw asymmetry or malocclusion in hemifacial microsomia. 7, 6
Follow-Up Schedule
First Year of Life
Audiologic reassessment every 3 months to monitor for progression of hearing loss and optimize amplification settings as ear canal acoustics change with growth. 5
Otolaryngology follow-up every 3-6 months to monitor for otitis media with effusion, which can compound conductive hearing loss and requires prompt treatment. 5
Speech-language evaluation at 6,12, and 18 months to track language milestones and adjust intervention intensity. 5
Ages 1-5 Years
Audiologic assessment every 6 months with behavioral audiometry (visual reinforcement audiometry, then conditioned play audiometry) to refine hearing thresholds and validate amplification benefit. 5
Annual speech-language evaluation to monitor vocabulary, syntax, and speech production relative to age-matched peers. 5
Plastic surgery consultation at age 3-4 years to begin discussing reconstruction options and timing with the family. 1, 3, 4
School Age and Beyond
Annual audiologic assessment to monitor for late-onset sensorineural hearing loss, which can occur in syndromic cases. 5
Plastic surgery follow-up every 6-12 months during the reconstruction process (typically ages 6-10 years), then annually to monitor for late complications such as framework exposure or asymmetry. 1, 6
Speech-language reassessment as needed based on academic performance and teacher/parent concerns. 5
Critical Pitfalls to Avoid
Do not delay amplification while awaiting CT results or genetic testing, as every month without auditory input during the first year of life increases the risk of permanent language deficits. 5
Do not perform auricular reconstruction before age 6 years, as inadequate rib cartilage volume leads to smaller, less durable frameworks and higher revision rates. 1, 4, 6
Do not overlook middle ear effusions in the contralateral normal ear, as even mild conductive overlay on the "good side" significantly impairs binaural hearing and should be treated aggressively with tympanostomy tubes. 5
Do not assume unilateral microtia requires no intervention—children with unilateral conductive hearing loss have documented deficits in sound localization, speech perception in noise, and academic performance, warranting consideration of amplification. 5
Do not perform atresiaplasty in cases with unfavorable anatomy (Jahrsdoerfer score <7), as the risk of complications outweighs the likelihood of achieving functional hearing. 3, 4
Do not neglect genetic evaluation, as identifying a syndrome alters surveillance protocols (e.g., renal ultrasound in branchio-oto-renal syndrome, cardiac echo in CHARGE syndrome) and provides recurrence risk counseling for future pregnancies. 5, 7, 8