{
  "abstract": "Introduction Systemic Sclerosis (SSc) is a multiorgan systemic autoimmune disease with widespread micro-vasculopathy and inflammation and fibrosis. Audiovestibular complications with resultant hearing loss is a rare manifestation of SSc.Material and Methods A 69-year lady with stable lcSSc (diagnosed 1990’s) with raynauds, telangiectasias, calcinosis, digital ulcers, upper and lower GI symptoms, stable mild CTD-ILD and late pattern NVC and with the background history of previous left breast cancer (treated in1996) and a stable obstructive airways disease presented with subacute hearing loss. Her medications were fluoxetine, Domperidone, cholecalciferol, omeprazole, sildenafil, Pregabalin, vit B12 injections. She reported in mid last year bilateral hearing loss (HL) with intermittent tinnitus.Results Her laboratory tests and ECHO and PFT’s are as tabulated ( table 1).She was assessed by the ENT team and had pure tone audiometry (figure 1) showing sloping to moderately severe sensorineural hearing loss (SNHL) bilaterally. There was asymmetry between the two ears at 4 and 6 kHz. Speech audiometry revealed excellent scores bilaterally (consistent with pure tone thresholds). She had Type A tympanogram bilaterally indicative of normal eardrum movement. She underwent MRI acoustic/temporal region and the retrococlear lesion, tumours, and specific local causes were ruled out. Trial of hearing aid was suggested thereafterConclusions Most ear complications are not noticeable or unreported. The presenting symptoms can range from fullness sensation, otalgia, hyperacusis, tinnitus and hearing loss usually sensorineural type (with good speech discrimination – like in our patient). The site of impairment usually is cochlea, however extracochlear auditory pathway can be involved. Mechanism is thought to be inflammation epineural vessels or vasa vasorum of cochlear nerve. There is decreased number of the spiral ganglion neurons in cochlea. SNHL is usually bilateral and symmetrical. Middle ear involvement is also suspected in some cases which includes narrowing of the incudomalleolar and incudostapaedial joint spaces with decrease in ossicular cartilage secondary to altered blood supply to the ossicles and cartilage.Individual cases of improvement of HL have been described after immunosuppressive agents (ISA), but due to very small sample groups with heterogenous results nil definite conclusion can be drawn.Assessment of hearing loss with pure tone audiometry, stapedial reflex threshold, speech audiometry, brainstem audiometry reflex, acoustic emissions and the treatment remains an unmet need.It warrants further research for the prevalence and predictable factors for potential HL. Also, the impact of ISA on hearing deficits and the longitudinal follow up developments once HL establishes needs to be determined to ascertain amenability to treatment.Abstract P.363 Figure 1Showing the pure tone audiometry test resultAbstract P.363 Table 1Laboratory & salient other investigation results",
  "authors": [
    {
      "affiliations": [
        "Te Whatu Ora Health NZ Waikato (Dept of Rheumatology), Hamilton, New Zealand",
        "Waikato School of Medicine, Hamilton, New Zealand",
        "Apollo Hospitals (AHERF), New Delhi, India"
      ],
      "name": "Kamal Solanki"
    },
    {
      "affiliations": [
        "Te Whatu Ora - Hauora a Toi Bay of Plenty (Dept of General Medicine & Rheumatology), Tauranga, New Zealand"
      ],
      "name": "Magdalina Budziakowska"
    }
  ],
  "title": "P.363 Uncommon but important presentation in systemic sclerosis - sensorineural hearing loss (SNHL",
  "uid": "32864162-58c6-53e6-989f-79396ee70886"
}