Ear Deformity

Occasionally we will see reports of children with Ear deformity(s) on the preadoption medical information.

Common terms

Some terms you may see:

Simple or Cup Ear: This is typically a cosmetic or appearance issue where the ear(s) are missing some of the normal folds and usually does not require intervention unless the patient or family electively (by choice) wishes to have a cosmetic revision.

Ear tags/pits: The majority of these are also typically cosmetic. Ear tags can be easily surgically removed, if this is preferred. Ear pits are little dimples that can get infected and if so, would need surgical revision. However most do not cause problems and would not need intervention. Studies are mixed but we typically recommend a renal ultrasound for children with ear tags or pits as there can be 8%1 associated kidney malformation. Some studies do not recommend routine renal ultrasound,2,3 however without a family history in adoption and especially if other anomalies are present (clefting, heart defects etc) we do recommend to investigate further when the child comes home.

Atresia: The external ear canal (outside part) and some of the inside parts of the ear are not present, did not develop.

Microtia: Small or underdeveloped outer ear. Very frequently associated with atresia. This is graded according to a (Marx) classification system.4

Microtia and Atresia are the issues that typically have greater follow-up needs. Multiple studies have been done on various populations with microtia/atresia. This issue is rare but does happen in about 1.84/10,000 live births.5 More males are affected than females.6 Multiple risk factors have been proposed including a vascular (blood vessel) accident that happens before birth, drug exposure, higher number of prior pregnancies, older age of the parent, genetics, as well as other factors.5,7,8 All children with microtia/atresia will require formal audiology hearing testing when they come to their new home as hearing loss is a possibility, especially with children who have major deformity of their ear(s).

About 60% of the time, microtia/atresia is non-syndromic meaning it is not associated with other physical/brain problems or genetic mutations.9,10 A simple, non-syndromic unilateral (one-sided) ear anomaly has some risk of decreased or no hearing for the ear that is affected but could have normal hearing on the non-affected/normal side. If there is atresia (the ear canal is not open), there will be some associated degree of hearing loss, but the majority of the time the inner ear functions normally. The better the outer ear is developed, the better the chances that the middle ear structures are present and may be functional.11 Depending upon the degree of development throughout the external auditory canal, middle ear, and inner ear, hearing can be rehabilitated or enhanced through various methods including surgery, hearing aids and bone anchored devices. Microtia (underdeveloped outer ear) typically requires either a prosthetic ear or some form of reconstruction when the child is school age. Often, reconstruction involves the use of skin and either rib cartilage or other materials to create a cosmetically similar ear structure, typically broken up into multiple stages.12,13

The main question with ear deformities is whether the ear is a marker for other problems. Children can have non-syndromic bilateral (both ears) microtia/atresia but when both ears have deformities, our experience has shown a higher rate of syndromes in these children as well as possible full hearing loss since both sides are affected. About 40% of the time, ear deformities are associated with other problems in the body and/or a syndrome.9,10 Dozens of associated medical problems have been described but when microtia/atresia is seen, the most common issues are listed below:5,14

  • Facial clefting: 12.4%
  • Cardiac defects: 12.1%
  • Small/missing eye: 5.6%
  • Limb (arm/leg) defects: 4.4%
  • Major kidney defects: 3.1%

One of the syndromes that has been associated with ear anomalies is oculo-auriculo-vertebral spectrum (OAVS) which includes Goldenhar syndrome and Hemifacial Microsomia (HFM). Branchio-oto-renal syndrome is also a syndrome that we have seen in preadoption information. With any syndrome, there are more organ systems that may be affected including the heart, kidneys, spine brain or other parts of the body.

Once home, children ideally need evaluations by their adoption specialist to help coordinate the care and referrals they will need. These children will need hearing and vision formally tested with audiology and ophthalmology. If available in your area, multidisciplinary craniofacial clinics can be especially helpful to provide support with genetics, plastic surgeons, ENT and other specialists in one clinic, to help the child be successful in their new family.

Summary

  • Ear "deformity": Seen occasionally in the preadoption referral information
  • Screening is recommended with a knowledgeable medical provider prior to adoption to assess the labs, physical exam, and any other imaging or medical information to look for associated syndromes.
  • One ear with deformity can be associated with some hearing loss in that ear.
  • Both ears with deformity can be associated with hearing loss in both ears, and is a stronger indicator of possible genetic or other syndromes that may affect other parts of the body and brain.
  • The child will likely need further studies/screening/workup when they come home.

References

  1. Kohelet D, Arbel E. A prospective search for urinary tract abnormalities in infants with isolated preauricular tags. Pediatrics. 2000;105(5):e61-e61.
  2. Fırat Y, Şireci Ş, Yakıncı C, et al. Isolated preauricular pits and tags: is it necessary to investigate renal abnormalities and hearing impairment? European Archives of Oto-Rhino-Laryngology. 2008;265(9):1057-1060.
  3. Kugelman A, Tubi A, Bader D, Chemo M, Dabbah H. Pre-auricular tags and pits in the newborn. J Pediatr. 2002;141(3):388-391.
  4. Marx H. Die Missbildungen des Ohres In: Denker A, Hahler O (eds): Handbuch der Hals-, Nasen-und Ohrenheilkunde mit einschluss der Grenzgebiete. Vol VI. . 1926.
  5. Harris J, Källén B, Robert E. The epidemiology of anotia and microtia. J Med Genet. 1996;33(10):809-813.
  6. Shaw GM, Carmichael SL, Kaidarova Z, Harris JA. Epidemiologic characteristics of anotia and microtia in California, 1989–1997. Birth Defects Research Part A: Clinical and Molecular Teratology. 2004;70(7):472-475.
  7. CASTILLA EE, ORIOLI IM. Prevalence rates of microtia in South America. Int J Epidemiol. 1986;15(3):364-368.
  8. Mastroiacovo P, Corchia C, Botto LD, Lanni R, Zampino G, Fusco D. Epidemiology and genetics of microtia-anotia: a registry based study on over one million births. J Med Genet. 1995;32(6):453-457.
  9. Llano-Rivas I, del Castillo V, Reyes R, Carnevale A. Microtia: a clinical and genetic study at the National Institute of Pediatrics in Mexico City. Arch Med Res. 1999;30(2):120-124.
  10. Eavey RD. Microtia and significant auricular malformation. Ninety-two pediatric patients. Arch Otolaryngol Head Neck. 1995;121(1):57-62.
  11. Kountakis SE, Helidonis E, Jahrsdoerfer RA. Microtia grade as an indicator of middle ear development in aural atresia. Archives of Otolaryngology—Head & Neck Surgery. 1995;121(8):885.
  12. Jiang H, Pan B, Lin L, Cai Z, Zhuang H. Ten-year experience in microtia reconstruction using tissue expander and autogenous cartilage. Int J Pediatr Otorhinolaryngol. 2008;72(8):1251-1259.
  13. Brent B. Microtia repair with rib cartilage grafts: a review of personal experience with 1000 cases. Clin Plast Surg. 2002;29(2):257-271.
  14. Suutarla S, Rautio J, Ritvanen A, Ala-Mello S, Jero J, Klockars T. Microtia in Finland: comparison of characteristics in different populations. Int J Pediatr Otorhinolaryngol. 2007;71(8):1211-1217.

  

Your feedback is important to us!  

Please take a moment to fill out this survey about Health Topics for Preadoptive Parents

 

Book Author

Judith K. Eckerle, M.D. 
Director, Adoption Medicine Clinic
Assistant Professor of Pediatrics



Co-author

Daniel Jethanamest, MD
Assistant Professor, Otolaryngology
Division of Otology, Neurotology and Skull Base Surgery
NYU Langone Medical Center

Provide feedback on this chapter