Why Can’t My Patient Hear Me?. James M. Sosman, MD. ID: The patient is a 57 year-old healthy Caucasian man who presented with intermittent headaches with sudden hearing loss. History of Present Illness:
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Why Can’t My Patient Hear Me? James M. Sosman, MD
ID:The patient is a 57 year-old healthy Caucasian man who presented with • intermittent headaches with sudden hearing loss. • History of Present Illness: • He noted a one month history of intermittent, throbbing occipital headaches that were not associated with photophobia, nausea, or vomiting. • There was no response to Ibuprofen and a moderate response to Tramadol. • He was admitted to a local hospital when the headaches began to be associated with jaw pain
HPI (continued)…: • At the local hospital, he complained of a left-sided earache with hearing loss, left-sided facial pain, severe rotational vertigo, nausea, vomiting, and visual “floaters” • A head CT scan was negative • He was transferred to UWHC for further evaluation.
Past Medical History: • History of “elevated liver enzymes” found incidentally 6 months ago • when he applied for life insurance; no further evaluation had • been done • Pinning of an open fracture of 5th digit at MCP joint on left arm • Inguinal hernia repair in the 1960s • Family/Social History: • Works as a farmer • No tobacco, alcohol, or illicit drug use. • Family history was negative for cancer and liver disease.
Medications: • None, besides occasional Ibuprofen and Tramadol for his recent headaches • Allergies: • Iodine contrast (nausea, vomiting, and diaphoresis) • ROS: • Low back pain for which he was treated with Valdecoxib • History of oral ulcers shortly after starting the Valdecoxib, this medication • was then stopped • No fevers, chills , night sweats , weight loss • No abdominal pain , diarrhea , constipation , melena , BRBPR • No rashes
Physical Examination: • Vitals – T 36.2 HR 64 BP 150/84 RR 16 SaO2 93% on RA • His sclera were anicteric. • The pupils were normal size with normal reaction to • accommodation and light. Fundus without lesions. • Visual acuity was normal and extra-ocular muscles were intact. • Mild horizontal nystagmus. • Otoscopic exam was normal. • The oropharynx was normal without any oral lesions • The temporal arteries were not tender to palpation • The neck was supple. There was no lymphadenopathy
Physical Examination: • The lungs had no wheezes, rhonchi, or crackles. • There were no murmurs, rubs, or extra sounds on cardiovascular • exam. • The abdomen was soft, non-tender, and non-distended. There were no masses and no hepatosplenomegaly. • The extremities were warm and there were no rashes or edema. • He had 2+ peripheral pulses in all extremities • The musculoskeletal exam was normal. • The cranial nerves were normal. Sound lateralized to the right with the Weber test. In both ears, air conduction was greater than bone conduction with the Rinne test
DDx: Unilateral Sensorineural Hearing Loss with or without Headache • meningitis • Meniere’s Disease • mastoiditis • viral infection – VZV (Ramsay Hunt), HSV 1, mumps, measles, rubella, influenza • Syphilis, Lyme disease • multiple sclerosis • autoimmune – RA, SLE, Sjögren’s, Antiphospholipid syndrome • vasculitis – Wegener’s, PAN, GCA • medication (drug toxicity) – Aminoglycosides, Furosemide, ASA Vincristine, Cisplatin, Vancomycin, Erythromycin, Quinine,
Laboratory Tests: • CBC WBC 11.1K Hct 35.5MCV 80 Plts 440K • Chem 7 Na 137 K 3.8 Chl 101 CO2 21 BUN 12 Cr 1 Glu 86 • Liver Function Tests Tbili 0.5 Alk Phos 523 GGT 1231 AST 23 ALT 37 • INR 1.0 Albumin 3.7 • TSH Normal • VDRL Non-reactive • Lumbar Puncture WBC 0-1 RBC 65 Glu 61 Pro 63 Culture Neg • ANA 1:160, speckled • Rheumatoid Factor 1:160 • ESR/CRP 116 / 5.3
Further Diagnostic Workup: • MRI/MRA of head was normal • Temporal Artery biopsy was negative for inflammation and negative • for multinucleated giant cells • Workup of abnormal liver function tests: • Hepatitis A/B/C serologies were negative • Ceruloplasmin was negative • alpha-1 anti-trypsin level was normal • anti-smooth muscle antibody was negative • c-ANCA was negative • p-ANCA was positive
Further diagnostic workup continued: • Liver biopsy was performed Pathologic evaluation revealed a lymphoplasmocytic portal infiltrate with focal inflammation of the bile ducts, but no actual loss of the bile ducts.
Further diagnostic workup continued: • Workup of sensorineural hearing loss: • Pure tone audiometry testing showed normal hearing on the • right and a severe to profound sensorineural hearing loss • on the left with no word recognition. • Vestibular testing revealed a unilateral vestibulopathy in • addition to the sensoirneural hearing loss diagnosed with • audiometry. • Workup of visual “floaters”: • Slit-lamp exam revealed a mild cell reaction in the anterior • chambers consistent with a mild bilateral anterior uveitis
Further diagnostic workup continued: • Workup of microcytic anemia (Hct 35.5, MCV 80) : • Iron Studies • Iron 43 (50-150 µg/dL) • TIBC 419 (250-370 µg/dL) • Ferritin 69.6 (15-400 ng/mL) • Stool guaiacs were positive • Further diagnostic tests?
Further diagnostic workup continued: So, a colonoscopy was performed… • The colonoscopy showed patchy erythema, edema, and granularity in the sigmoid colon extending to the cecum with sparing of the rectum and mid-transverse colon as well as a normal terminal ileum
Further diagnostic workup continued: • Biopsy of the right colon revealed glandular architectural distortion and • a diffuse lymphocytic inflammatory infiltrate consistent with • chronic colitis
Differential Diagnosis: • Colonscopy revealed chronic colitis • Ophthalmology exam revealed anterior uveitis • Neuro-Otologic exam identified a senorineural hearing loss • The abnormal liver function tests were thought to be secondary to an • autoimmune cholangitis (based on the histopathology of the liver • biopsy and the response to steroid treatment) Presumptive Diagnosis: subclinical inflammatory bowel disease (UC) with extra-intestinal manifestations including autoimmune sensorineural hearing loss.
Autoimmune Sensorineural Hearing Loss and IBD: • Extraintestinal manifestations of IBD are common and occur in up to • 25% of patients with Crohn’s and Ulcerative Colitis • Extraintestinal manifestations include: • reactive arthropathy (up to 20% of patients) • axial arthropathy • uveitis and episcleritis • skin lesions (Erythema nodosum and Pyoderma gangrenosum) • primary sclerosing cholangitis (2-5% of patients) • sensorineural hearing loss (<5%) • Autoimmune sensorineural hearing loss (ASNHL) was first described • in 1979 by McCabe who reported 18 patients with progressive bilateral • hearing loss that responded to steroids and/or cyclophosphamide
ASNHL and IBD continued: • ASNHL has been reported in a small number of patients with UC and • Crohn’s, it is usually bilateral and sudden in onset • Hearing loss usually occurs during active IBD, but there • have been reports of ASNHL occurring when the disease is in remission. • Other immune-mediated conditions associated with SNHL include: • Rheumatoid Arthritis (30-55%) • Sjögren’s (25-40%) • Polyarteritis nodosa • SLE (21%) • Relapsing polychondritis • Giant Cell Arteritis • Wegener’s granulomatosis (8%)
ASNHL and IBD continued: • Kumar et al. in 2000 reported results from a prospective study of 20 • patients with active UC and 20 healthy age and sex-matched • controls: • pure tone audiometry revealed significant SNHL throughout • all frequencies in patients with UC compared with controls • no UC patients or controls complained of auditory symptoms • no association was found between hearing loss and smoking • history, extent of disease, coexistence of other extraintestinal • manifestations, or immunosuppressive therapy • subclinical SNHL can be associated with UC
ASNHL: • More common in women than men • Pathogenesis of ASNHL is unclear, hypotheses include: • T-lymphocyte mediated cytotoxicity • immune complex deposition • vasculitis of the inner ear • auto-immunity due to hidden self-antigens • cross-reacting antibodies • non-specific effect of systemic inflammation with increased • levels of peripheral blood T-lymphocytes and circulating • pro-inflammatory cytokines such as IL-1, IL-6, and TNF • which can cause a low-grade progressive tissue injury • Diagnosis is clinical based on presentation and response to therapy
ASNHL continued: • Specifically, antibodies to type II collagen have been implicated as well • as an anti-68 kd autoantibody. The 68 kd antigen has in turn been • linked to the highly inducible heat shock protein (hsp) 70. Antibodies to hsp 70 can be identified using the Otoblot Western blot assay (Sensitivity = 42%, Specificity = 90%, PPV 91%) • hsp 70 is one of the many cross-reacting proteins against the inner • ear which may be linked to immune-mediated hearing loss • Therefore, the clinical response to steroid therapy establishes the • presumptive diagnosis of immune-mediated hearing loss • In order to prevent irreversible damage and help preserve auditory • function immediate treatment with steroids is recommended
Hospital Course: • The patient was started on Fluromethalone drops for his uveitis which • completely resolved within two weeks. • Therapy for the patient’s sensorineural hearing loss included • Prednisone 1 mg/kg with a quick taper, but repeat audiometric testing • showed normalization of the vestibulopathy with only minimal • improvement in his hearing. He continues to have some dysequilibrium • and tinnitus. • His liver function tests normalized after initiating oral steroids. • Throughout his hospital stay he continued to deny any GI complaints. • However, five months later… he is reporting 2-3 loose stools • per day with occasional blood. A repeat colonoscopy is pending.
Learning Objectives: • Identify extraintestinal manifestations of IBD. • Recognize that there is a well-documented association between • sensorineural hearing loss and IBD. • Learn some of the hypotheses behind the pathogenesis of autoimmune • sensorineural hearing loss. • Recognize the importance of immediate treatment of autoimmune • sensorineural hearing loss.
References: • Bachmeyer C, Leclerc-Landgraf N, Laurette F, Coutarel P, Cadranel JF, Medioni J, • Dhote R, Mougeot-Martin M. Acute autoimmune sensorineural hearing loss • associated with Crohn’s disease. Amer Jour Gastro. Vol 93 (12): 2565-2567, 1998. • 2. Kanra G, Kara A, Secmeer G, Ozen H, Gurakan F, Akcoren Z, Atas A. • Sensorineural hearing loss as an extra-intestinal manifestation of ulcerative • colitis in an adolescent girl with pyoderma gangrenosum. Eur J Pediatr. • Vol 161: 216-218, 2002. • 3. Kumar BN, Smith MSH, Walsh RM, Green JRB. Sensorineural hearing loss in • ulcerative colitis. Clin Otolaryngol Vol 25: 143-145, 2000. • 4. McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol. Vol 88: 585-9, • 1979. • 5. Mathews J, Rao S, Kumar BN, Phil M. Autoimmune sensorineural hearing • loss: is it still a clinical diagnosis? Jour Laryngology & Otology.Vol 117: 212-214, • 2003.