sign in sign up
<<
Home , Deafness, Hearing loss, Vertigo

Self-assessment corner 375 Postgrad Med J: first published as 10.1136/pgmj.74.872.375 on 1 June 1998. Downloaded from , loss, and

S Ali Raza, James J Phillipps

A 39-year-old Caucasian man presented to his general practitioner with an episode of vertigo, right-sided and tinnitus. He was treated with . Two weeks later, he also experienced left-sided hearing loss with tinnitus and was hospitalised. There were no other oto- logical symptoms. His medical history included ulcerative colitis for three years and he was tak- ing sulphasalazine. There were no prior problems. Otoneurological examination did not show any abnormality apart from the obvious hearing loss (figure 1). Magnetic resonance imaging of the internal acoustic meati and cerebellopontine angles was normal. Routine haematological and biochemical profiles showed that the total plasma protein and its IgG fraction were significantly increased and the erythrocyte sedimentation rate (ESR) was 38 mm/h.

Frequency (Hz) Frequency (Hz) 125 250 500 1000 2000 4000 8000 125 250 500 1000 2000 4000 8000 -10 -10 0 _ 0 _ 10 _ 10 _ 20 20 > 30 - , 30 - 40 I 40 D 50 c] 50 c - 60 2 60 - ° 70 - - 70- 0, *c 80 .c 80 ) 90 Co 90 _ I 100 _ I 100 _ 110 _ 110 _ 120 120 Department of 130 130 _ Otolaryngology, 140 140 http://pmj.bmj.com/ Singleton Hospital, Figure 1 Presentation Swansea, Wales, UK S Ali Raza J J Phillipps

Correspondence to Mr S Ali Questions Raza, Specialist Registrar in Otolaryngology, Royal 1 What type of hearing loss is apparent on the audiogram on September 28, 2021 by guest. Protected copyright. Gwent Hospital, Newport, (figure 1) ? Gwent NP9 2UB, UK 2 Suggest the possible differential diagnoses in this case. 3 What systemic can be associated with this type of hearing loss ? Accepted 19 November 1997 4 What is the recommended treatment and prognosis ? 376 Self-assessment corner

Answers Discussion

QUESTION 1 mediated audiovestibular

Immunologically Postgrad Med J: first published as 10.1136/pgmj.74.872.375 on 1 June 1998. Downloaded from The audiogram in figure 1 shows sensorineural disorders are a rare clinical entity. The diagno- hearing loss. This type of hearing loss can be sis is dependent upon a high index of clinical caused by diseases of the sensory organ, the suspicion and early recognition is crucial to or its neural connections, ie, the acous- successful treatment. Like any other tissue of tic nerve and its central pathways. the body, the can sometimes be involved in a localised or generalised auto- QUESTION 2 immune reaction. Different mechanisms de- There are clinical features of a progressive sen- scribed in the literature include both cell- sorineural hearing loss. It can be associated mediated and antibody-mediated pathways, with viral , vascular occlusion, coch- vasculitis involving the inner ear blood vessels, lear membrane breaks, autoimmune inner ear involvement of the endolymphatic sac and stria disorders, , acoustic neu- vascularis, anticollagen II antibodies and roma, ototoxic drugs and psychogenic causes. mechanisms against the central audiovestibular Bilateral otological symptoms, no evidence of pathways.2-5 It has also been suggested that this ototoxic drug intake, a history of ulcerative form of loss be associated colitis and results of various investigations hearing may with make the diagnosis of immune-related hearing inheritance of certain human leucocyte anti- loss more in this case. gens, when increased frequencies of Cw7, Cw4 likely and B35 and decreased frequency of DR4 was noticed in such cases.6 QUESTION 3 Certain auto-immune disorders have been The inner ear is one of the places in the known to be associated with sensorineural human body where a tissue sample cannot be hearing loss. These include rheumatoid arthri- obtained routinely for immune testing. Some tis, Sjogren syndrome, Wegner's granulomato- specific tests have been described, which sis, polyarteritis nodosa, juvenile chronic ar- involve the use of inner ear tissue obtained thritis, systemic lupus erythematosus and from patients undergoing acoustic Cogan syndrome. Sensorineural hearing loss surgery. For humoral immunity, the presence has also been reported to be associated with of cross-reacting antibodies determined by ulcerative colitis.' Although, the exact aetiology indirect immunofluorescence or immunoper- ofulcerative colitis is still unknown, an immune oxidase techniques indicates autoimmune dis- cause is strongly suspected due to extra- ease. To determine cell-mediated immunity, intestinal manifestations like arthritis, peri- leucocyte migration inhibition and lymphocyte cholangitis and pyoderma gangrenosum. Other transformation tests involve incubating the indirect evidence for the immune aetiology is patient's mononuclear leucocytes with the provided by the therapeutic effect of immuno- inner ear tissue extracts. A response is consid- suppressant drugs. ered positive when migration or transformation is significantly different from those of controls http://pmj.bmj.com/ QUESTION 4 containing medium alone.7 These specific tests The treatment of immune-mediated hearing are expensive and require inner ear antigens. loss includes steroids and cytotoxic agents. The Therefore, they are mainly tools at latter include cyclophosphamide, methotrexate present. Various non-specific immunological and azathioprine. Prognosis is good when the tests are freely available. These include ESR, treatment is instituted early in the course of serum immunoglobulins, auto-antibodies,

. In resistant cases or when immunosup- serum immune complexes and complement on September 28, 2021 by guest. Protected copyright. pressant therapy is contra-indicated, plas- assay.8 These tests are inexpensive and helpful mapheresis may be considered. when positive. In our case, the high level of

Frequency (Hz) Frequency (Hz) 125 250 500 1000 2000 4000 8000 125 250 500 1000 2000 4000 8000 -IU I I ! I in. 0- 10 - 10 20 20 ' 30 -J 30 m 40 I 40 _1 9 50 50 n 60- 60 - 070 CO0 70 C 80 0 o 90- co K I 100 - I 110

120 -

130 -

140 - Figure 2 Post-treatment audiogram Self-assessment corner 377

serum immunoglobulins and IgG led us to sus- pect the auto-immune nature of the problem. Features suggestive of The immune-mediated loss can immune-mediated loss hearing hearing Postgrad Med J: first published as 10.1136/pgmj.74.872.375 on 1 June 1998. Downloaded from present as a solitary event or as a part of a more auto-immune disorder. It is * sensorineural hearing loss in early and middle generalised usually aged adults] [for whom no other cause can be bilateral and symmetrical; vertigo and tinnitus found may or may not be present. Since there is no * slowly progressive sensorineural hearing loss characteristic presentation, such a diagnosis (weeks or months rather than hours or days) should be considered in a patient presenting * sensorineural hearing loss in a person with an with any combination of these symptoms. Cer- already existing auto-immune condition tain clinical features should raise the alarm (box).7 Steroids and immunosuppressant therapy are the mainstay of treatment. We used in presentation and unavailability of a specific azathioprine and obtained a good response. diagnostic test. However, a thorough clinical Fluctuations in hearing thresholds were ob- examination and non-specific laboratory tests served during the initial adjustment of the ster- are needed in all suspicious cases. oid dose. Subsequently, the hearing remained improved to a useful level, despite gradual Final diagnosis tapering of the steroids (figure 2). In conclu- sion, immune-mediated hearing loss is one of Immune-mediated sensorineural loss. the few forms ofsensorineural that are hearing treatable by medical means.9 The diagnosis can Keywords: auto-immune disease; sensorineural hear- sometimes be difficult due to a wide variation ing loss; ulcerative colitis

1 Hollanders D. Sensorineural deafness - a new 6 Bowman CA, Nelson RA. Human leukocytic antigens in of ulcerative colitis? Postgrad MedJ 1986;62:753-5. autoimmune sensorineural hearing loss. Laryngoscope 1987; 2 Veldman JE. Immune-mediated inner ear disorders: an 97:7-9. otoimmunologist's view. Adv Otorhinolaryngol 1991;46:71-7. 7 Hughes GB, Kinney SE, Barna BP, Calabrese LH. Practical 3 Helfgott SM, Mosciscki RA, Martin JS, et al. Correlation versus theoretical between antibodies to type II collagen and treatment management of autoimmune inner ear outcome in bilateral progressive sensorineural hearing loss. disease. Laryngoscope 1984;94:758-67. Lancet 1991;337:387-9. 8 Zanetti D, Franceschini F, Antonelli AR. Immunological 4 Leone CA, Feghali JG, Linthicum FH. Endolymphatic sac: investigation for sensorineural hearing loss in the otologic possible role in autoimmune sensorineural hearing loss. Ann clinic: preliminary reports. Acta Otorhinolaryngol Belg 1994; Otol Rhinol Laryngol 1984;93:208-9. 48:71-9. 5 Salomon P, Charachon R, Lejeune JM. Indirect immun- 9 McCabe BF. Autoimmune inner ear disease: results of ofluorescence in the investigation of rapidly progressive sen- therapy. Adv Otorhinolaryngol 1991;46:78-81. sorineural hearing loss and Meniere's disease. Acta Otolaryngol (Stockh) 1993;113:318-20. http://pmj.bmj.com/ Hypercalcaemia and abdominal

David Scott-Coombes, Andrew Williams on September 28, 2021 by guest. Protected copyright.

A previously fit 75-year-old woman presented to Accident and Emergency with a 12- hour his- tory of constant upper abdominal pain. Clinical examination revealed pallor but no and guarding in the epigastrium. Her biochemical results were serum amylase 2878 IU/1 (normal <90), bilirubin 33 umol/l (3-20), alkaline phosphatase 42 IU/l (30-150), aspartate transaminase 354 IU/1 (10-50), albumin 38 g/l (35-50), calcium 2.85 mmol/l (2.2-2.6). Department of Questions Surgery, King's College Hospital, 1 What is differential for the abdominal London SE5 9RS, UK your diagnosis pain? D Scott-Coombes 2 Comment on the relationship between the serum calcium and amylase levels. A Williams 3 What further investigations would you request? 4 How would you further investigate and manage the hypercalcaemia if it were due to primary Accepted 21 January 1998 hyperparathyroidism?