| Santarelli et al.(55. Santarelli R, Monte PSED, Arslan EGE. Auditory neuropathy in systemic sclerosis: a speech perception and evoked potential study before and after cochlear implantation. Eur Arch Otorhinolaryngol. 2006;263:809-15.) |
Insidious onset of symptoms, with progressive difficulty to understand the speech |
Slightly higher auditory thresholds 1 year after the onset of the disease, and significant aggravation after 2 years; normal tympanometry and absence of bilateral acoustic reflexes; bilateral absence of speech intelligibility; DPOAE at high frequencies, and absence in low frequencies 2 years after the onset of the disease; normal vestibular evaluation; after cochlear implant, 70% of speech recognition |
| Teasdall et al.(99. Teasdall RD, Frayaha RA, Schulman LE. Cranial nerve involvement in systemic sclerosis (scleroderma): a report of 10 cases. Medicine (Baltimore). 1980;59(2):149-59.) |
Of the 10 patients, 3 presented tinnitus |
Bilateral HS in 2 of 10 patients |
| Berrettini et al.(1010. Berrettini S, Ferri C, Pitaro N, Bruschini P, Latorraca A, Sellari-Franceschini S, et al. Audiovestibular involvement in systemic sclerosis. ORL. 1994;56:195-8.) |
Of the 37 patients, 11 complained of tinnitus; 4 complained of vestibular dysfunctions; and 8 complained of auditory and vestibular dysfunctions |
SNHL in 10 and mixed HL in 4 cases; unilateral HL in one case; in patients with mixed HL, acoustic immittance findings indicate tympanosclerosis; two of them presented positional nystagmus; one had unilateral vestibular dysfunction, and one had bilateral deficit after caloric stimulation |
| Kastanioudakis et al.(1111. Kastanioudakis I, Ziavra N, Politi EN, Exarchakos G, Drosos AA, Skevas A. Hearing loss in progressive systemic sclerosis patients: a comparative study. Otolaryngol Head Neck Surg. 2001;124(5):522-5.) |
Not reported |
Six of them had SNHL, and one had mixed HL; out of these, one belonged to group A, one to group B, four to group C, and one to group D; 10% of these had bilateral eustachian tube pathology; vocal audiometry compatible with cochlear disorder and tympanometry without changes |
| Amor-Dorado et al.(1212. Amor-Dorado JC, Barreira-Fernandez MP, Arias-Nuñez MC, Gomez-Acebo A, Llorca J, Gonzalez-Gay MA. Benign paroxysmal positional vertigo and clinical test of sensory interaction and balance in systemic sclerosis. Otol Neurotol. 2008;29(8):1155-61.) |
Not reported |
Seven were diagnosed with BPPV; two patients with limited SS had involvement of the posterior semicircular canal; three with limited SS, and two with diffuse SS presented compromised horizontal semicircular canal; changes in sensory integration tests observed in 20 patients |
| Maciaszczyk et al.(1313. Maciaszczyk K, Waszczykowska E, Pajor A, Bartkowiak-Dziankowska B, Durko T. Hearing organ disorders in patients with systemic sclerosis. Rheumatol Int. 2011;31(11):1423-8.) |
Of the 20 patients, 60% reported vertigo, 55% had headaches, 50% had tinnitus, 40% had hyperacusis, 40% had hearing loss, and 30% had auricular plenitude |
Eight had SNHL, all were women aged between 42 and 77 years; two had unilateral SNHL; mild for three of them, and moderate for the rest. Five of them presented acoustic reflex, one did not, and two had absence in frequencies of 2 or 4 kHz; BAEP had no changes |
| Bassyouni et al.(1414. Bassyouni IH, Emad Y, Rafaat HA, Dabbous AO. Relationship between nailfold capillary abnormalities and vestibular dysfunction in systemic sclerosis. Joint Bone Spine. 2011;78(3):266-9.) |
Not reported |
Vestibular dysfunction in 33% of the sample; no relationship was observed between vestibular compromise and age, duration and other clinical parameters of the disease |
| Abou-Taleb and Linthicum(1515. Abou-Taleb A, Linthicum FH Jr. Scleroderma and hearing loss: (histopathology of a case). J Laryngol Otol. 1987;101(7):656-62.) |
Progressive auditory loss |
Bilateral mixed HL; 68% speech discrimination in the right ear; 78% in the left ear |
| Deroee et al.(1616. Deroee AF, Huang TC, Morita N, Hojjati M. Sudden hearing loss as the presenting symptom of systemic sclerosis. Otol Neurotol. 2009;30(3):277-9.) |
Sudden bilateral hearing loss, especially at the left |
High-frequency SNHL in the right ear direita and severe HL in the left ear; 100% speech discrimination in the right ear and 0% in the left ear; normal bilateral tympanometry; 1 year after treatment for SS, improved HL and neuropathy, and increasing speech discrimination — to 68% in the left ear |
| Amor-Dorado et al.(1717. Amor-Dorado JC, Arias-Nuñez MC, Miranda-Filloy JA, Gonzalez-Juanatey C, Llorca J, Gonzalez-Gay MA. Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies. Medicine (Baltimore). 2008;87(3):131-41.) |
Of the 35 patients, 19 (54%) reported HL |
HL in 27 patients, being 24 with SNHL (11 asymmetric and 13 symmetric), and 3 with mixed HL; 13 had flat configuration and 14 had high-frequency loss; 2 had unilateral HL; 2 had absent bilateral acoustic reflex, and 11 had unilateral; 10 presented abnormal oculocephalic response, and 9, positional nystagmus; changes in caloric testing and clinical sensory integration tests were found in 31 and 46% of the patients, respectively |
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