• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
Logo of geneticsGeneticsCurrent IssueInformation for AuthorsEditorial BoardSubscribeSubmit a Manuscript
Genetics. Mar 2004; 166(3): 1385–1393.
PMCID: PMC1470800

Heritability and segregation analysis of deafness in U.S. Dalmatians.

Abstract

Hereditary loss of hearing affects many breeds of the domestic dog, but the Dalmatian has the highest prevalence. Approximately 30% are affected in the United States (U.S.) population. It is widely accepted that a relationship exists between deafness and pigmentation in the dog and also in other animals. While the Dalmatian exemplifies this relationship, the genetic origin and mode of inheritance of deafness in this breed are unknown. The goals of this study were to: (1) estimate the heritability of deafness in an extended kindred of U.S. Dalmatians and (2) determine, through complex segregation analysis, whether there is a major segregating locus that has a large effect on the expression of deafness. A kindred of 266 Dalmatians was assembled, of which 199 had been diagnosed using the brainstem auditory evoked response to determine auditory status. Of these, 74.4% (N = 148) had normal hearing, 18.1% (N = 36) were unilaterally deaf, and 7.5% (N = 15) were bilaterally deaf. A heritability of 0.73 was estimated considering deafness a dichotomous trait and 0.75 considering it as a trichotomous trait. Although deafness in the Dalmatian is clearly heritable, the evidence for the presence of a single major gene affecting the disorder is not persuasive.

Full Text

The Full Text of this article is available as a PDF (107K).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Bonney GE. Regressive logistic models for familial disease and other binary traits. Biometrics. 1986 Sep;42(3):611–625. [PubMed]
  • Brenig B, Pfeiffer I, Jaggy A, Kathmann I, Balzari M, Gaillard C, Dolf G. Analysis of the 5' region of the canine PAX3 gene and exclusion as a candidate for Dalmatian deafness. Anim Genet. 2003 Feb;34(1):47–50. [PubMed]
  • Cargill Edward J, Clark Leigh Anne, Steiner Jörg M, Murphy Keith E. Multiplexing of canine microsatellite markers for whole-genome screens. Genomics. 2002 Sep;80(3):250–253. [PubMed]
  • Elston RC, Namboodiri KK, Glueck CJ, Fallat R, Tsang R, Leuba V. Study of the genetic transmission of hypercholesterolemia and hypertriglyceridemia in a 195 member kindred. Ann Hum Genet. 1975 Jul;39(1):67–87. [PubMed]
  • Famula TR, Oberbauer AM, Sousa CA. Complex segregation analysis of deafness in Dalmatians. Am J Vet Res. 2000 May;61(5):550–553. [PubMed]
  • Famula TR, Oberbauer AM, Williams DC. Gender effects in hearing loss in Dalmatians. Prev Vet Med. 2001 Jan 17;48(1):15–24. [PubMed]
  • Greenberg DA. The effect of proband designation on segregation analysis. Am J Hum Genet. 1986 Sep;39(3):329–339. [PMC free article] [PubMed]
  • Holliday TA, Nelson HJ, Williams DC, Willits N. Unilateral and bilateral brainstem auditory-evoked response abnormalities in 900 Dalmatian dogs. J Vet Intern Med. 1992 May-Jun;6(3):166–174. [PubMed]
  • HUDSON WR, RUBEN RJ. Hereditary deafness in the Dalmatian dog. Arch Otolaryngol. 1962 Mar;75:213–219. [PubMed]
  • Jarvik GP. Complex segregation analyses: uses and limitations. Am J Hum Genet. 1998 Oct;63(4):942–946. [PMC free article] [PubMed]
  • Juraschko K, Meyer-Lindenberg A, Nolte I, Distl O. Analysis of systematic effects on congenital sensorineural deafness in German Dalmatian dogs. Vet J. 2003 Sep;166(2):164–169. [PubMed]
  • Kay R, Palmer AC, Taylor PM. Hearing in the dog as assessed by auditory brainstem evoked potentials. Vet Rec. 1984 Jan 28;114(4):81–84. [PubMed]
  • Mair IW. Hereditary deafness in the dalmatian dog. Arch Otorhinolaryngol. 1976 Feb 8;212(1):1–14. [PubMed]
  • Marshall AE. Brain stem auditory-evoked response of the nonanesthetized dog. Am J Vet Res. 1985 Apr;46(4):966–973. [PubMed]
  • Marshall AE. Use of brain stem auditory-evoked response to evaluate deafness in a group of Dalmatian dogs. J Am Vet Med Assoc. 1986 Apr 1;188(7):718–722. [PubMed]
  • Morton NE, MacLean CJ. Analysis of family resemblance. 3. Complex segregation of quantitative traits. Am J Hum Genet. 1974 Jul;26(4):489–503. [PMC free article] [PubMed]
  • Muhle Anne C, Jaggy A, Stricker C, Steffen F, Dolf G, Busato A, Kornberg M, Mariscoli M, Srenk P, Gaillard C. Further contributions to the genetic aspect of congenital sensorineural deafness in Dalmatians. Vet J. 2002 May;163(3):311–318. [PubMed]
  • Strain GM. Congenital deafness and its recognition. Vet Clin North Am Small Anim Pract. 1999 Jul;29(4):895–vi. [PubMed]
  • Strain George M. Deafness prevalence and pigmentation and gender associations in dog breeds at risk. Vet J. 2004 Jan;167(1):23–32. [PubMed]
  • WAARDENBURG PJ. A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness. Am J Hum Genet. 1951 Sep;3(3):195–253. [PMC free article] [PubMed]
  • Steel KP. Inherited hearing defects in mice. Annu Rev Genet. 1995;29:675–701. [PubMed]
  • Steel KP, Bussoli TJ. Deafness genes: expressions of surprise. Trends Genet. 1999 Jun;15(6):207–211. [PubMed]
  • Steel KP, Kros CJ. A genetic approach to understanding auditory function. Nat Genet. 2001 Feb;27(2):143–149. [PubMed]
  • Steel KP, Barkway C, Bock GR. Strial dysfunction in mice with cochleo-saccular abnormalities. Hear Res. 1987;27(1):11–26. [PubMed]

Articles from Genetics are provided here courtesy of Genetics Society of America

Formats:

Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...

Links

  • MedGen
    MedGen
    Related information in MedGen
  • PubMed
    PubMed
    PubMed citations for these articles