Myelin protein P0 has been identified as an autoantigen in inner ear diseases. In order to study autoimmune hearing loss, we performed brain stem auditory-evoked potential (BAEP) studies on P0-sensitized mice. Two P0-sensitized mice showed hunched posture, poor coat, loss of body weight, and abnormal walking with a waddling gait. About 25% of the P0-sensitized mice developed hearing loss. In the BAEP study, peak latencies of waves I, III, and V and the interpeak latency I-III were prolonged in the P0-sensitized hearing loss group of mice. Hearing thresholds were elevated in this group of mice in comparison with the control mice. Inflammatory cell infiltration was observed in the cochlear nerve region, and a reduced number of spiral ganglion cells was also detected. These results suggest that P0-sensitized mice are a useful model for studying autoimmune inflammation of the peripheral portion of the auditory system.
JoliatTSeyerJBernsteinJ. Antibodies against a 30 kilodalton cochlear protein and type II and IX collagens in the serum of patients with inner ear diseases. Ann Otol Rhinol Laryngol1992; 101: 1000–6.
KosakaKYamanobeSTomiyamaS-IYagiT. Inner ear autoantibodies in patients with sensorineural hearing loss. Acta Otolaryngol Suppl (Stockh) 1995(suppl 519): 176–7.
4.
SuzukiMKrugMSChengK-CYazawaYBernsteinJYooT-J. Antibodies against inner-ear proteins in the sera of patients with inner-ear diseases. ORL1997; 59: 10–7.
5.
CaoM-YGersdorffMDeggoujNWarnyMTomasiJ-P. Detection of inner ear disease autoantibodies by immunoblotting. Mol Cell Biochem1994; 146: 157–63.
6.
CaoMYDeggoujNGersdorffMTomasiJ-P. Guinea pig inner ear antigens: extraction and application to the study of human autoimmune inner ear disease. Laryngoscope1996; 106: 207–12.
7.
CaoMYDupriezVJRiderMH. Myelin protein P0 as a potential autoantigen in autoimmune inner ear disease. FASEB J1996; 10: 1635–40.
8.
GreenfieldSBrostoffSEylarEHMorellP. Protein composition of myelin of the peripheral nervous system. J Neurochem1973; 20: 1207–16.
9.
IshaqueARoomiMWSzymanskaIKowalskiSEylarEH. The P0 glycoprotein of peripheral nerve myelin. Can J Biochem1980; 58: 913–21.
10.
FilbinMTWalshFSTrappBDPizzeyJATennekoonGI. Role of myelin P0 protein as a homophilic adhesion molecule. Nature1990; 344: 871–2.
11.
LiningtonCLassmannHOzawaKKosinSMonganL. Cell adhesion molecules of the immunoglobulin supergene family as tissue-specific autoantigens: induction of experimental allergic neuritis (EAN) by P0 protein—specific T cell lines. Eur J Immunol1992; 22: 1813–7.
12.
LemkeGAxelR. Isolation and sequence of a cDNA encoding the major structural protein of peripheral myelin. Cell1985; 40: 501–8.
13.
SakamotoYKitamuraKYoshimuraKNishijimaTUyemuraK. Complete amino acid sequence of P0 protein in bovine peripheral nerve myelin. J Biol Chem1987; 262: 4208–14.
14.
HayasakaKNanaoKTaharaM. Isolation and sequence determination of cDNA encoding the major structural protein of human peripheral myelin. Biochem Biophys Res Commun1991; 180: 515–8.
15.
KulkensTBolhuisPAWoltermanRA. Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie-Tooth disease type 1B. Nat Genet1993; 5: 35–9.
16.
Satya-MurtiSCacaceATHansonPA. Abnormal auditory evoked potentials in hereditary motor-sensory neuropathy. Ann Neurol1979; 5: 445–8.
17.
RaglanEPrasherDKTrinderERudgeP. Auditory function in hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease). Acta Otolaryngol (Stockh) 1987; 103: 50–5.
MilnerPLovelidgeCATaylorWAHughesRAC. P0 myelin protein produces experimental allergic neuritis in Lewis rats. J Neurol Sci1987; 79: 275–85.
21.
WaksmanBHAdamsRD. A comparative study of experimental allergic neuritis in the rabbit, guinea pig and mouse. J Neuropathol Exp Neurol1956; 15: 293–332.
22.
TerayamaYSasakiY. Studies on experimental allergic (isoimmune) labyrinthitis in guinea pigs. Acta Otolaryngol (Stockh) 1963; 58: 49–64.
ArchelosJJRoggenbuckKSchneider-SchauliesJLiningtonCToykaKVHartungH-P. Production and characterization of monoclonal antibodies to the extracellular domain of P0. J Neurosci Res1993; 35: 46–53.
27.
UyemuraKSuzukiMKitamuraK. Studies on myelin proteins in human peripheral nerve. Adv Exp Med Biol1978; 100: 95–115.
28.
WhitakerJN. The protein antigens of peripheral nerve myelin. Ann Neurol1981; 9(suppl): 56–64.
29.
SpiesJMPollardJDBonnerJGWestlandKWMcLeodJG. Synergy between antibody and P2-reactive T cells in experimental allergic neuritis. J Neuroimmunol1995; 57: 77–84.
30.
ZhangX-MEschTRClarkL. Neuritogenic Lewis rat T cells use Tcrb chains that induce a new Tcrb-V8 family member. Immunogenetics1994; 40: 266–70.
31.
GregorianSKClarkLHeber-KatzEAmentoEPRostamiA. Induction of peripheral tolerance with peptide-specific anergy in experimental autoimmune neuritis. Cell Immunol1993; 150: 298–310.
32.
Khalili-ShiraziAHughesRACBrostoffSWLiningtonCGregsonN. T cell responses to myelin proteins in Guillain-Barré syndrome. J Neurol Sci1992; 111: 200–3.
33.
AdelmannMLiningtonC. Molecular mimicry and the autoimmune response to the peripheral nerve myelin P0 glycoprotein. Neurochem Res1992; 17: 887–91.
34.
BollensenESteckAJSchachnerM. Reactivity with the peripheral myelin glycoprotein P0 in serum from patients with monoclonal IgM gammopathy and polyneuropathy. Neurology1988; 38: 1266–70.
35.
OnishiSManabeT. A guide to objective audiometry using averaging techniques. Kyoto, Japan: Kimpodo, 1976: 76–94.
