Familial Occurrence of Unilateral Vestibular Schwannoma

Vestibular schwannoma (VS) may present clinically in one of two forms: sporadic unilateral or hereditary bilateral. Almost all cases of familial transmission have been associated with the diagnosis of neurofibromatosis type II (NF‐2). In this report, we describe nine families (18 individuals) presen...

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Bibliographic Details
Published in:The Laryngoscope 1997-09, Vol.107 (9), p.1176-1180
Main Authors: Bikhazi, Nadim B., Slattery, William H., Lalwani, Anil K., Jackler, Robert K., Bikhazi, Paul H., Brackmann, Derald E.
Format: Article
Language:English
Subjects:
Adult
Age Factors
Aged
Biological and medical sciences
Cranial Nerve Neoplasms - genetics
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Female
Follow-Up Studies
Gene Expression
Genetic Variation
Genomic Imprinting
Hearing Disorders - etiology
Humans
Infectious Disease Transmission, Vertical
Male
Medical sciences
Middle Aged
Neurilemmoma - genetics
Neurofibromatosis 2 - genetics
Neuroma, Acoustic - genetics
Otorhinolaryngology. Stomatology
Phenotype
Postural Balance
Probability
Retrospective Studies
Sensation Disorders - etiology
Sex Factors
Tinnitus - etiology
Tumors
Vertigo - etiology
Vestibular Nerve - pathology
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Summary:Vestibular schwannoma (VS) may present clinically in one of two forms: sporadic unilateral or hereditary bilateral. Almost all cases of familial transmission have been associated with the diagnosis of neurofibromatosis type II (NF‐2). In this report, we describe nine families (18 individuals) presenting with unilateral VS without evidence of NF‐2. In four of the nine families, the affected individuals were of parent‐offspring relationship, in three families they were cousin‐cousin, and in the remaining two families, they were sibling‐sibling and aunt‐nephew. No other members of the families were diagnosed with NF‐2. There was no evidence for gender predilection or genomic imprinting among affected individuals. This study suggests that familial occurrence of unilateral VS may be genetically inherited as it occurs more commonly than would be estimated by chance alone. Future genetic studies will elucidate whether occurrence of unilateral VS in these families represents a variable expression of NF‐2, chance occurrence of unilateral VS in families, or a new genetic disorder.
ISSN:0023-852X
1531-4995
DOI:10.1097/00005537-199709000-00004