Hereditary deafness in the Dalmatian dog

Summary

The temporal bones from 18 deaf Dalmatian dogs were examined with the light microscope, and the cochleo-saccular degeneration process studied. End organ degeneration is already advanced by the age of 4 weeks, whilst loss of neurons in the spiral ganglion was found only in adult animals. Both processes are first evident in the central portion of the cochlea, and increase in extent with advancing age. The implications of these findings for the management of non-conductive hearing loss in man are discussed.

This is a preview of subscription content, access via your institution.

References

  1. Alexander, G., Tandler, J.: Untersuchungen an kongenital tauben Hunden, Katzen und an jungen kongenital tauben Katzen. Arch. Ohrenheilk. 66, 161–179 (1905)

    Google Scholar 

  2. Altmann, F.: Histologic picture of inherited nerve deafness in man and animals. Arch. Otolaryng. 51, 852–890 (1950)

    Google Scholar 

  3. Anderson, H., Henricson, B., Lundquist, R. G., Wedenberg, E., Wersäll, J.: Genetic hearing impairment in the Dalmatian dog. Acta oto-laryng. (Stockh.) Suppl. 232, 1–32 (1968)

    Google Scholar 

  4. Axelsson, A., Ernstson, S.: Cochlear vascular anatomy in a strain of the waltzing guinea pig. Acta otolaryng. (Stockh.) 74, 172–182 (1972)

    Google Scholar 

  5. Bosher, S. K., Hallpike, C. S.: Observations on the histological features, development and pathogenesis of the inner ear degeneration of the deaf white cat. Proc. roy. Soc. B 162, 147–170 (1965)

    Google Scholar 

  6. Bredberg, G.: Cellular pattern and nerve supply of the human organ of Corti. Acta oto-laryng. (Stockh.) Suppl. 236, 1–135 (1968)

    Google Scholar 

  7. Brown, K. S., Bergsma, D. R., Barrow, M. V.: Animal models of pigment and hearing abnormalities in man. In: Birth defects: original article series, vol. VII, no. 4 (ed. D. Bergsma), pp. 102–109. Baltimore: Williams and Wilkins Comp. 1971

    Google Scholar 

  8. Corti, A.: Recherches sur l'organe de l'ouie des mammiferes. Z. Wiss. Zool. 3, 109–169 (1851)

    Google Scholar 

  9. Deol, M. S.: The anomalies of the labyrinth of the mutants Varitint-Waddler, Shaker-2 and Jerker in the mouse. J. Genet. 52, 562–588 (1954)

    Google Scholar 

  10. Deol, M. S.: A gene for uncomplicated deafness in the mouse. J. Embryol. exp. Morph. 4, 190–195 (1956)

    Google Scholar 

  11. Deol, M. S.: The relationship between abnormalities of pigmentation and of the inner ear. Proc. roy. Soc. Lond. A 175, 201–217 (1970)

    Google Scholar 

  12. Duvall, A. J., III, Ward, W. D., Lauhala, K. E.: Stria ultrastructure and vessel transport in acoustic trauma. Ann. Otol. (St. Louis) 83, 498–514 (1974)

    Google Scholar 

  13. Ernstson, S.: Cochlear morphology in a strain of the waltzing guinea pig. Acta oto-laryng. (Stockh.) 71, 469–482 (1971)

    Google Scholar 

  14. Foss, I.: Stria vascularis and Reissner's membrane of the hereditary deaf white Norwegian Dunkerhound. J. Ultrastruct. Res. 25, 162–163 (1968)

    Google Scholar 

  15. Foss, I.: Stria vascularis and Reissner's membrane of hereditary deaf white mink. Exp. Cell Res. 58, 452 (1969)

    Google Scholar 

  16. Gacek, R.: The pathology of hereditary sensorineural hearing loss. Ann. Otol. (St. Louis) 80, 289–298 (1971)

    Google Scholar 

  17. Gacek, R. R., Rasmussen, G. L.: Fiber analysis of the statoacoustic nerve of quinea pig, cat, and monkey. Anat. Rec. 139, 455–463 (1961)

    Google Scholar 

  18. Guild, S. R.: A graphic reconstruction method for the study of the organ of Corti. Anat. Rec. 22, 141–157 (1921)

    Google Scholar 

  19. Hawkins, J. E., Jr.: Comparative otopathology: aging, noise and ototoxic drugs. Adv. Oto-Rhino-Laryng. 20, 125–141 (1973)

    Google Scholar 

  20. Howe, H. A.: The reaction of the cochlear nerve to destruction of its end organs: a study on deaf albino cats. J. comp. Neurol. 62, 73–79 (1935)

    Google Scholar 

  21. Hudson, W. R., Ruben, R. J.: Hereditary deafness in the Dalmatian dog. Arch. Otolaryng. 75, 213–219 (1962)

    Google Scholar 

  22. Hughson, W., Thompson, E., Whiting, E. G.: An experimental study of bone conduction. Ann. Otol. (St. Louis) 45, 844–858 (1936)

    Google Scholar 

  23. Igarashi, M., Alford, B. R., Saito, R., Cohn, A. M., Watanabe, T.: Inner ear anomalies in dogs. Ann. Otol. (St. Louis) 81, 249–255 (1972)

    Google Scholar 

  24. James, T. N.: Congenital deafness and cardiac arrhythmias. Amer. J. Cardiol. 19, 627–643 (1967)

    Google Scholar 

  25. Johnsson, L.-G., Hawkins, J. E., Jr., Muraski, A. A., Preston, R. E.: Vascular anatomy and pathology of the cochlea in Dalmatian dogs. In: Vascular disorders and hearing defects (ed. A. J. D. de Lorenzo), pp. 249–293. Baltimore: University Park Press 1973

    Google Scholar 

  26. Konigsmark, B. W.: Syndromal approaches to the nosology of hereditary deafness. In: Birth defects: original article series, vol. VII, no. 4 (ed. D. Bergsma), pp. 2–17. Baltimore: Williams and Wilkins Comp. 1971

    Google Scholar 

  27. Lindsay, J. R.: Profound childhood deafness. Ann. Otol. (St. Louis) Suppl. 5, 1–121 (1973)

    Google Scholar 

  28. Lurie, M. H.: The membranous labyrinth in the congenitally deaf collie and Dalmatian dog. Laryngoscope (St. Louis) 58, 279–287 (1948)

    Google Scholar 

  29. Mair, I. W. S.: Hereditary deafness in the white cat. Acta oto-laryng. (Stockh.) Suppl. 314, 1–48 (1973)

    Google Scholar 

  30. Mitchell, A. L.: Dominant dilution and other color factors in collie dogs. J. Hered. 26, 425–430 (1935)

    Google Scholar 

  31. Nachlas, N. E., Lurie, M. H.: The stria vascularis: review and observations. Laryngoscope (St. Louis) 61, 989–1003 (1951)

    Google Scholar 

  32. Rawitz, B.: Gehörorgan und Gehirn eines weißen Hundes mit blauen Augen. Morphol. Arbeiten 6, 545–554 (1896)

    Google Scholar 

  33. Schuknecht, H. F.: Neuroanatomical correlates of auditory sensitivity and pitch discrimination in the cat. In: Neural mechanisms of the auditory and vestibular systems (ed. G. L. Rasmussen, W. F. Windle), pp. 76–90. Springfield: C. C. Thomas 1960

    Google Scholar 

  34. Schuknecht, H. F., Igarashi, M., Gacek, R. R.: The pathological types of cochleo-saccular degeneration. Acta oto-laryng. (Stockh.) 59, 154–167 (1965)

    Google Scholar 

  35. Sorsby, A., Davey, J. B.: Ocular associations of dappling (or merling) in the coat colour of dogs. I. Clinical and genetical data. J. Genet. 52, 425–440 (1954)

    Google Scholar 

  36. Spoendlin, H.: Degeneration behaviour of the cochlear nerve. Arch. klin. exp. Ohr.-, Nas.- u. Kehlk.- Heilk. 200, 275–291 (1971)

    Google Scholar 

  37. Spoendlin, H.: Innervation densities of the cochlea. Acta oto-laryng. (Stockh.) 73, 235–248 (1972)

    Google Scholar 

  38. Spoendlin, H.: Neuroanatomy of the cochlea. In: Facts and models in hearing (ed. E. Zwicker, E. Terhardt), pp. 18–32. Berlin-Heidelberg-New York: Springer 1974

    Google Scholar 

  39. Spoendlin, H.: Retrograde degeneration of the cochlear nerve. Acta oto-laryng. (Stockh.) 79, 266–275 (1975)

    Google Scholar 

  40. Suga, F., Hattler, K. W.: Physiological and histopathological correlates of hereditary deafness in animals. Laryngoscope (St. Louis) 80, 80–104 (1970)

    Google Scholar 

  41. Sugiura, A., Hilding, D. A.: Cochleo-saccular degeneration in Hedlund white mink. Acta oto-laryng. (Stockh.) 69, 126–137 (1970)

    Google Scholar 

  42. Talley, J. N.: An examination of the hearing mechanism of the behaviorally deaf dalmatian. Graduate College of the University of Illinois: Thesis, pp. 1–45 (1965)

  43. Waardenburg, P. J.: A new syndrome combining developmental abnormalities of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness. Amer. J. hum. Genet. 3, 195–253 (1951)

    Google Scholar 

  44. Wright, S.: Color inheritance in mammals. J. Hered. 9, 87–90 (1918)

    Google Scholar 

Download references

Author information

Affiliations

Authors

Additional information

The author would like to express his appreciation and gratitude for the assistance and advice he has received from Dr. C. Fernandez, University of Chicago, Chicago, Illinois.

This study was supported in part by Grant NB-0682 from the National Institute of Health, Washington, and by Grant C.27. 14-2 from the Norwegian Research Council for Science and the Humanities.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Mair, I.W.S. Hereditary deafness in the Dalmatian dog. Arch Otorhinolaryngol 212, 1–14 (1976). https://doi.org/10.1007/BF00456358

Download citation

Key words

  • Hereditary deafness
  • Dalmatian dogs
  • Cochleo-saccular degeneration
  • Spiral ganglion degeneration