Syndromes with Congenital Hearing Loss

  • Peter D. Phelps
  • Glyn A. S. Lloyd


Many syndromes are associated with deafness and an even greater number with abnormalities of the pinna — usually a real or apparent low position on the side of the head. These combinations of deformities are well described in various reference books (Gorlin et al. 1976; Konigsmark and Gorlin 1976). However, while there are some specific clusters of anomalies many, or perhaps most, of these children do not fit the description of any particular syndrome. There may be associated congenital heart disease and also abnormalities of the genito-urinary system. Potentially serious renal anomalies may go undetected in early life and it has been suggested that all children with malformed ears should have an intravenous pyelogram (Rapin and Ruben 1976).


Hearing Loss Temporal Bone Osteogenesis Imperfecta Oval Window External Auditory Meatus 
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Copyright information

© Springer-Verlag London 1990

Authors and Affiliations

  • Peter D. Phelps
    • 1
  • Glyn A. S. Lloyd
    • 2
    • 3
  1. 1.Walsgrave HospitalRoyal National Throat, Nose and Ear HospitalLondonUK
  2. 2.Department of RadiologyRoyal National Throat, Nose and Ear HospitalLondonUK
  3. 3.Moorfields Eye HospitalLondonUK

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