International Journal of Clinical Case Reports 2013, Vol.3, No.7, 37
-
38
http://ijccr.sophiapublisher.com
37
Research Report Open Access
Bilateral Microtia: A Rare Case
Brijendra Singh ¹ , Renu Gupta ² , Rajneesh Garg ³ , Sunil K Khattri
4
1
Department of Anatomy, All India Institute of Medical Sciences, Jodhpur, 342005, India
2
Department of Anatomy, All India Institute of Medical Sciences, Jodhpur, 342005, India
3
Department of Orthopedics, S.N. Medical College, Jodhpur, 342005, India
4
Director Rajiv Gandhi Superspeciality Hospital, Tahirpur, New Delhi, India
Corresponding author email:
drbrijendrasingh@gmail.com
International Journal of Clinical Case Reports 2013, Vol.3, No.7 doi: 10.5376/ijccr.2013.03.0007
Received: 12 May, 2013
Accepted: 20 May, 2013
Published: 22 May, 2013
Copyright: © 2013 Singh et al., This is an open access article publ ished under the terms of the Creative Commons Attribution License, which permits unrestricted
use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article as:
Singh et al., 2013, Bilateral Microtia: A Rare Case, International Journal of Clinical Case Reports, Vol.3, No.7 37-38 (doi: 10.5376/ijccr.2013.03.0007)
Abstract
Anotia is a condition when ears are completely absent while microtia is a congenital anomaly of the face in which ear ar e
malformed since birth that ranges in severity from mild structural abnormalities to complete absence of the ear and its parts. It occurs as
an isolated birth defect or as a part of spectrum of anomalies or a syndrome. This may happen as a part of first branchial arch syndrome
or in is olation too. The prevalence is considered to be high er in Hispanics, Asians, Native Americans, and Andeans. The etiology of
microtia and the causes of this congenital anomaly is still not clearly defined or explained till now; there is wide variability in prevalence
that is poorly understood too. Strong evidence supports the role of environmental and genetic causes for microtia. In this paper we tried
to review current knowledge of the epidemiology and genetics of microtia, including potential candidate genes supported by evidence
from human syndromes and animal models. Because our findings are entirely different about its hereditical and bilateral involvement
makes this case a rare and special.
Keywords
Microtia, Anotia, Congenital defect, Hereditary, Ear deformity
Introduction
The ear is composed of three parts: the internal,
middle and external portions. Microtia is a congenital
anomaly, characterized by a narrow, blocked or absent
ear canal. Microtia can affect one ear ( unilateral) or
both ears (bilateral). It occurs in every 1 out 6 000 to
12 000
births. The right ear is m ore commonly
affected than left and it is more common in male sex
as compared to female sex. On basis of anatomical
involvement microtia may be cl assified in grades
(
Meurman, 1957).
Classification
Grade 1: A smaller version of a typical sized ear, still
having same physical characteristic of a typical sized
ear and c ontaining a small but present external ear
canal.
Grade 2: A partially formed outer ear with very small
or narrow ear canal. The ear canal may be very narrow
or closed (canal stenosis) producing a conductive
hearing loss.
Grade 3: Absence of external ear with a small peanut
shaped structure (some cartilage with mostly ear lobe)
and an absence of the external ear canal and ear drum
(
known as aural atresia).
Grade 4: Absence of the complete ear (anotia).
In most instances the children with microtia disorders
will have normal middle and inner ears, making a
cosmetic condition rather than a typical cause of nerve
deafness.
In this paper we discussed a case of m icrotia on one
side and anotia on otherside.
Case report
A rare case of microtia was observed during OPD
hours in Genetic Counseling Clinic at HAHC Hospital
at Hamdard Institute of Medical Sciences and Resea-
rch, Jamia Hamdard, New Delh i. A child was born
with microtia (grade 3) on right side and a malformed
external ear on t he left side. On ri ght side there was
absence of external ear w ith small peanut s haped
structure and absence of external ear canal (Figure 1).
While on opposite side a large lobulated external ear
was there with stenosis in ear c anal (grade 2) see
(
Figure 2).
No one of either parents or their family members were
having any congenital malformation or defects inclu-