Skip to main content
Log in

Hearing loss in facioscapulohumeral dystrophy

  • Original Investigations
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Bilateral sloping high frequency hearing loss of 20–90 dB was found in six out of ten patients with infantile or adolescent onset FSHD. In all cases the basic defect could be traced to the cochlea. The outer hair cells of the basal turn are predominantly affected. In 20 patients with various other forms of muscular dystrophy or neuromuscular disorders with an FSH distribution, no sensorineural hearing loss was found. Myopathology of FSHD patients extended from mild to severe, often showing inflammatory infiltrates and type I fibre atrophy, without unequivocal differences between the two groups with and without hearing loss. It is concluded that cochlear dysfunction is a specific and frequenct phenomenon of early onset FSHD.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

FSH:

facioscapulohumeral

FSHD:

facioscapulohumeral dystrophy

MD:

muscular dystrophy

SISI:

short increment sensitivity index

References

  1. Allen NR (1973) Hearing acuity in patients with muscular dystrophy. Dec Med Child Neurol 15:500–505

    Google Scholar 

  2. Baiocco F, Testa D, d'Angelo A, Cocchini F (1984) Abnormal auditory evoked potentials in Déjerine-Sottas disease. J Neurol 231:46–49

    Google Scholar 

  3. Bethlem J, Van Wijngaarden GK, De Jong J (1973) The incidence of lobulated fibers in the facioscapulohumeral type of muscular dystrophy and the limb girdle syndrome. J Neurol Sci 18:351–358

    Google Scholar 

  4. Carroll JE, Brooke MH (1979) Infantile facioscapulohumeral dystrophy. In: Serratrice G, Roux H (eds) Peroneal atrophies and related disorders. Masson, New York, pp 305–318, Discussion pp 318–319

    Google Scholar 

  5. Duchenne GB (de Boulogne) (1862) Album de photographies pathologiques complémentaire du livre intitulé de l'électrisation localisée. JB Baillère et Fils, Paris London New York

    Google Scholar 

  6. Dubowitz V, Brooke MH (1973) Muscle biopsy: a modern approach. Saunders, London Philadelphia Toronto

    Google Scholar 

  7. Engel WK, Kossmann RJ (1963) Selective involvement of histochemical type I muscle fibers in a patient with facioscapulohumeral muscular dystrophy. Neurology 13:362

    Google Scholar 

  8. Fenichel GM, Emery ES, Hunt P (1967) Neurogenic atrophy simulating facioscapulohumeral dystrophy. Arch Neurol 17:257–260

    Google Scholar 

  9. Flock A, Cheung HC (1977) Actin filaments in sensory hairs of inner ear receptor cells. J Cell Biol 75:339–343

    Google Scholar 

  10. Goebel HH, Prange H, Gullotta F, Kiefer H, Jones MZ (1979) Becker's X-linked muscular dystrophy. Histological, enzymhistochemical, and ultrastructural studies of two cases, originally reported by Becker. Acta Neuropathol (Berl) 46:69–71

    Google Scholar 

  11. Hiraide F, Kodera K (1975) Sensori-neural deafness in two cases of progressive muscular atrophic diseases. Audiol Japan 18:208–213

    Google Scholar 

  12. Kousseff B, Gieron M, Korthals J, Nichols P (1983) Sensorineural hearing loss and facioscapulohumeral dystrophy. Poster, Birth Defects Conference, Washington (June 20)

  13. Landouzy L, Déjerine J (1885) De la myopathie atrophique progressive: myopathie sans neuropathie, débutant d'ordinaire dans l'enfance, par la face. Rev Méd 5:81–117, 253–366

    Google Scholar 

  14. Lenhardt E (1978) Praktische Audiometrie. Thieme, Stuttgart New York

    Google Scholar 

  15. Mastaglia FL, Walton J (eds) (1982) Skeletal muscle pathology. Churchill Livingstone, Edinburgh London

    Google Scholar 

  16. McGarry J, Garg B, Silbert S (1983) Death in childhood due to facio-scapulo-humeral dystrophy. Acta Neurol Scand 68:61–63

    Google Scholar 

  17. Meyerson MD, Lewis E, Karen I (1984) Facioscapulohumeral dystrophy and accompanying hearing loss. Arch Otolaryngol 110: 261–266

    Google Scholar 

  18. Mullendore JM, Stoud RJ (1961) Speech pattern of muscular dystrophic individuals. J Speech Hear Disord 26:252–257

    Google Scholar 

  19. Sarnat HB (1983) Muscle pathology and histochemistry. Am Soc Clin Pathologists Press, Chicago

    Google Scholar 

  20. Schmitt J, Barrucand D, Schmidt C (1977) De la maladie au syndrome de Landouzy-Déjerine. Evolution du concept de l'amyotrophie facio-scapulo-humérale. Rev Neurol (Paris) 133:279–282

    Google Scholar 

  21. Schubert W, Boxler K, Jerusalem F, Willeit J (1984) Neurogene Befunde bei facio-scapulo-humeralen Syndromen. Fortschr. Myologie 7:157–164

    Google Scholar 

  22. Small RG (1968) Coats' disease and muscular dystrophy. Trans Amer Acad Ophthalmol Otolaryngol 72:225–231

    Google Scholar 

  23. Taylor DA, Carroll JE, Smith ME, Johnson MO, Johnston GP, Brooke MH (1982) Facioscapulohumeral dystrophy associated with hearing loss and Coats syndrome. Ann Neurol 12:395–398

    Google Scholar 

  24. Thompson DS, Woodward JB, Ringel SP, Nelson LM (1983) Evoked potential abnormalities in myotonic dystrophy. Electroencephalogr Clin Neurophysiol 56:453–456

    Google Scholar 

  25. Wakayama Y, Takayanagi T, Mukoyama M, Sobue I (1976) A case of facioscapulohumeral myopathy with skeletal muscle and liver mitochondrial inclusion bodies. Clin Neurol (Tokyo) 16:706–714

    Google Scholar 

  26. Wulff JD, Lin JT, Kepes JJ (1982) Inflammatory facioscapulohumeral muscular dystrophy and Coats syndrome. Ann Neurol 12:398–401

    Google Scholar 

  27. Zenner HP (1981) Cytoskletal and muscle-like elements in cochlear hair cells. Arch Otorhinolaryngol 230:81–92

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Voit, T., Lamprecht, A., Lenard, H.G. et al. Hearing loss in facioscapulohumeral dystrophy. Eur J Pediatr 145, 280–285 (1986). https://doi.org/10.1007/BF00439401

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00439401

Key words

Navigation