Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management.

OBJECTIVES The objective of this study was to identify factors correlated with the CT outcome and to examine the contribution of the CT scan in the aetiological diagnosis and management of unilateral sensorineural hearing loss in childhood. METHODS The records of 35 consecutively investigated patients by the Audiology Department of Great Ormond Street Hospital between January 1996 and June 1998 were reviewed. The CT results, population sample characteristics, initiation of further investigations after the CT results and management decisions based on the CT results were tabulated and analysed. RESULTS In a series of 35 consecutively investigated children with unilateral sensorineural hearing loss, 11 CT scans were identified as abnormal. The CT findings were: labyrinthitis ossificans (3), unilaterally dilated vestibular aqueduct (2), bilaterally dilated vestibular aqueduct (2), unilateral deformity of the cochlea ('Mondini') (1), unilateral severe labyrinthine dysplasia (1), unilateral markedly narrow internal acoustic meatus (1), bilaterally dilated lateral semicircular canals (1). The presence of progressive hearing loss was a significant predictor of abnormal CT outcome, while the severity of hearing loss was not. The CT scans offered valuable information regarding the aetiological diagnosis in all cases and, in addition, prompted the appropriate vestibular rehabilitation in three cases, further investigations in four (with dilated vestibular aqueduct) and hearing preservation counselling in two (bilateral DVA) (seven out of 35 = 20%). CONCLUSION All children with unilateral sensorineural hearing loss should have a CT scan of the petrous pyramids/IAMs performed at some stage, as not only aetiology but also prognosis and management of these cases may be significantly influenced by the CT outcome.

[1]  S. Shott,et al.  Value of ct scan in the diagnosis of temporal bone diseases in children , 1986, The Laryngoscope.

[2]  J. Hartvig Jensen,et al.  Unilateral sensorineural hearing loss in children: cognitive abilities with respect to right/left ear differences. , 1989, British journal of audiology.

[3]  D. Edelstein,et al.  The large vestibular aqueduct syndrome in children. A review of 12 cases and the description of a new clinical entity. , 1989, Archives of otolaryngology--head & neck surgery.

[4]  G. Sutton,et al.  Risk factors for childhood sensorineural hearing loss in the Oxford region. , 1997, British journal of audiology.

[5]  A. Davis,et al.  Risk factors for hearing disorders: epidemiologic evidence of change over time in the UK. , 1995, Journal of the American Academy of Audiology.

[6]  Ó. Dias,et al.  Childhood deafness in Portugal--aetiological factors and diagnosis of hearing loss. , 1990, International journal of pediatric otorhinolaryngology.

[7]  L. Bilaniuk,et al.  Usefulness of computed tomographic scan in the evaluation of sensorineural hearing loss in children. , 1992, Archives of otolaryngology--head & neck surgery.

[8]  W. Reardon,et al.  Radiological malformations of the ear in Pendred syndrome. , 1998, Clinical radiology.

[9]  D. Watson,et al.  Auditory brainstem response screening for hearing loss in high risk neonates. , 1996, International journal of pediatric otorhinolaryngology.

[10]  J. A. Martin Aetiological factors relating to childhood deafness in the European community. , 1982, Audiology : official organ of the International Society of Audiology.

[11]  C. Cremers,et al.  The sex-ratio in childhood deafness, an analysis of the male predominance. , 1994, International journal of pediatric otorhinolaryngology.

[12]  M. Pembrey,et al.  Phenotypic manifestations of branchiootorenal syndrome , 1995 .

[13]  W. J. Kelly,et al.  Unilateral hearing loss in children , 1991, The Laryngoscope.

[14]  M. Feinmesser,et al.  Etiology of childhood deafness with reference to the group of unknown cause. , 1986, Audiology : official organ of the International Society of Audiology.

[15]  J. L. Culbertson,et al.  Identification, Assessment, and Management of Children with Unilateral Sensorineural Hearing Loss , 1986, Ear and hearing.

[16]  G. Everberg LIII Etiology of Unilateral Total Deafness Studied in a Series of Children and Young Adults , 1960 .

[17]  P. Marsella,et al.  Unilateral sensorineural hearing loss in children. , 1988, Scandinavian audiology. Supplementum.