Pediatric Ear Abnormalities

Sana Bhatti, MD Mississippi Center for Advanced Medicine Objectives

1. Understand the normal anatomy of the ear. 2. Identify common congenital ear abnormalities as they present in the neonatal period. 3. Recognize the psychosocial impact of ear differences on pediatric patients. 4. Facilitate prompt diagnosis of congenital ear abnormalities and refer patients to specialists so that non-surgical treatment can be initiated in the neonatal period. Normal Ear Anatomy

Superior Crus Helix Triangular fossa Scapha

Inferior Crus

Antihelix Tragus Concha Anti-Tragus

Lobule Congenital Ear Abnormalities

• Categorized as either: • Malformations – due to disrupted embryogenesis • Deformations – due to external forces • 15-20% Newborns • Can be mild and only affect the external ear • Can be associated with hearing loss, anomalies of other structures such as the jaw, orbit, nerves, muscles, soft tissues, kidneys. Ear Abnormalities

• Step 1: Diagnosis • Malformation • Deformation • Treatment • Timing • Referral to plastic surgeon Malformations

1. Anotia 2. Microtia 3. Cryptotia 4. Pre-auricular sinuses & remnants Microtia

1. External ear with absent skin or cartilage that is small, collapsed or only has an earlobe present 2. Can occur as an isolated birth defect, or as a part of a spectrum of anomalies or as a component of a syndrome. 3. Most often a/w conductive hearing loss 4. Prevalence varies geographically and is reported to be from 0.83 to 17.4 per 10,000 births • males (2 or 3:1 • unilateral (70-90%) • right-left-bilateral ratio is 6:3:1.26 Microtia Treatment

• Involves multidisciplinary approach • Restoration of hearing • Surgical reconstruction of the external ear • Initial treatment consists of an ABR, frequent ear evaluations (high risk for ear infections), renal ultrasound Microtia Surgery

• Refer to plastic surgeon early on • Higher prevalence of mood disorders • Ear reconstruction typically begins at age 6 or older Microtia Surgery • Several surgical options 1. Autologous 2. Composite reconstruction 3. Osteointegrated prosthesis Autologous

Osteointegrated Prothesis Composite reconstruction Cryptotia

• Hidden ear • Superior portion of ear buried underneath temporal skin • Treatment • Nonsurgical molding • Surgery involving release of superior ear and full thickness skin grafting for sulcus creation Pre-auricular Anomalies

• Pre-auricular sinuses and remnants • Screening renal ultrasounds not recommended • Do not regress overtime • Treatment • Sinuses- if infected, abx & surgical excision • Remnants- surgical excision Deformations

1. Stahl’s ear 2. Constricted ear 3. Prominent ear Management of Ear Deformations

• Nonsurgical correction can be made by forcing the ear cartilage into proper position and maintaining it there for several weeks • Should start in the first week of life • Molding can help correct deformation due to circulating maternal estrogen, which peak at 3 days of life and then normalize around 6 weeks of age Is the child eligible for ear molding?

• Stahl ear • Prominent ear • Lop ear • Helical rim abnormalities • Cryptotia What does ear molding look like? • Non-surgical, no anesthesia • Low risk • Biweekly visits • Typically takes 6 weeks to treat Ear Molding BEFORE AFTER BEFORE AFTER BEFORE AFTER What about older children?

• Many kids and adults grow up with ear anomalies without having ear molding as a baby • Self conscious, end up covering their ears with their hair • Sometimes bullied at school • Children, teenagers and adults have cartilage that is too stiff for molding and will require surgical correction, this is called an otoplasty, also known as ear pinning Otoplasty

• Common procedure for prominent ears • Surgical • General anesthesia for younger children and teenagers • General anesthesia, sedation or local anesthesia for adults • Outpatient procedure that usually takes about 2 hours • Post-operative care requires staying away from contact sports for 4-6 weeks What is an otoplasty? Treating Ear Diagnostic Ear Nonsurgical Renal Surgery Abnormality Hearing Correction Abnormalities US Malformation Microtia + + + - • Mild deformations affecting Cryptotia - - Possible + only the external ear Pre-auricular - + + - • Nonsurgical ear molding remnant Pre-auricular - - If infected - • Surgery for older children sinus and adults Deformation Prominent - - If not corrected + with molding • Microtia Stahl - - If not corrected + • Multi-disciplinary with molding craniofacial team Constricted - - If not corrected + with molding • Hearing aid ASAP • Surgical correction can begin as early as age 6