Characteristics of sensorineural hearing loss in children
with inner ear anomalies
James M. Coticchia, MD
, Abhay Gokhale
, Josh Waltonen, MD
, Baran Sumer, MD
Department of Otolaryngology Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Mich, USA
Tufts University School of Medicine, Northwestern University, Feinberg School of Medicine, Boston, Mass, USA
Department of Otolaryngology Head and Neck Surgery, Northwestern University, Feinberg School of Medicine, Chicago, Ill, USA
Department of Otolaryngology Head and Neck Surgery, Washington University School of Medicine, St Louis, Mo, USA
Received 18 February 2005
Purpose: To determine whether hearing loss in children with inner ear anomalies has some distinctive
characteristics when compared to children with hearing loss but without inner ear anomalies.
Methods: Temporal bone computed tomography scans of 69 patients with sensorineural hearing loss
were examined for inner ear abnormalities of which 17 were identified. The medical histories of
these patients were reviewed for the characteristics of their hearing loss, including initial
presentation, natural history, and nature of loss, as well as the family history of hearing loss and
risk factors for hearing loss. These were compared to age-matched controls with hearing loss but
without inner ear anomalies.
Results: Seventeen patients had inner ear anomalies. Records of 14 of these patients were compared
to patients without inner ear anomalies. Regarding age of onset, 71.4% of patients with anomalies
had onset of their hearing loss at less than 2 years old vs 78.6% without anomalies. Regarding
unilateral vs bilateral, 42.9% of patients with anomalies were unilateral vs 28.6% of patients without
anomalies. For patients with anomalies, 85.7% were stable and 14.3% were progressive; without
anomalies, 71.4% were stable, 21.4% were progressive, and 7.1% were fluctuating. Regarding
family history, only 14.3% of patients without anomalies had a positive family history vs 56% of
patients with anomalies.
Conclusions: Children with inner ear anomalies and sensorineural hearing loss have an increased
incidence of unilateral hearing loss and stable hearing loss as compared to controls with
sensorineural hearing loss without inner ear anomalies. In addition, children with inner ear
anomalies and sensorineural hearing loss are less likely to have a family history of hearing loss.
D 2006 Elsevier Inc. All rights reserved.
Congenital sensorineural hearing loss (SNHL) is a
relatively common diagnosis in children with incidence
rates ranging from 1:1000 to 1:2000 [1-3] . Prompt diagnosis
and treatment of congenital SNHL are essential as significant
developmental delays in speech and language can ensue.
Unfortunately, because of the variety of etiologies and
presentations of SNHL in pediatric populations, diagnosis
and initiation of proper treatment can be difficult. One
common attribute that appears in at least a proportion of
children with SNHL is an inner ear anomaly (IEA).
It is generally accepted that approximately 50% of
congenital hearing loss is attributed to genetics, 25% to
environmental factors such as perinatal infection, ototoxic
drug use, or trauma, and 25% is idiopathic [2,3]. Congenital
hearing loss due to genetic factors is often due to single-
gene mutations that can lead to manifestations that are either
syndromic or nonsyndromic, and are inherited in either an
autosomal dominant, autosomal recessive, X-linked, or
0196-0709/$ – see front matter D 2006 Elsevier Inc. All rights reserved.
T Corresponding author. Department of Otolaryngology Head and Neck
Surgery, Wayne State University, School of Medicine, 540 E. Canfield
Avenue, 5E-UHC, Detroit, MI 48202, USA. Tel.: +1 313 577 0805; fax: +1
313 577 8555.
E-mail address: firstname.lastname@example.org (J.M. Coticchia).
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 33 – 38