Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Earaches are common causes of fever, irritability, failure to feed, and vomiting in young children. Patients with sickle cell syndromes may be predisposed to serious complications such as meningitis so early, appropriate treatment is important. Sensorineural hearing loss (SNHL) has been associated with sickle cell disease (SCD) in older children and adults and may affect up to 40% of the population. Desferrioxamine used for chelation therapy for transfusion related iron overload can cause ototoxicity.

Subjective Data

Present Illness. Document onset of symptoms, frequency, headache, fever, chills, irritability, decreased appetite/feeding, hearing loss, vertigo, tinnitus, nausea, vomiting, pharyngitis, nasal congestion, snoring, ear, drainage, foreign body in ear canal (Q-tip, bobby pin, insect), trauma, swimming, pressure change, noise exposure. Desferrioxamine usage.

Past Medical History. Past history of ear infections or allergies. Family history of hearing loss, tinnitus, vertigo, or kidney disease. Present and recent medications and drug allergies.

• Vital Signs. Temperature, pulse, blood pressure.
• Head. Tenderness over mastoids, swelling, pre- or post-auricular adenopathy, palpate temporomandibular joint and parotid.
• Ear. Pain with pulling pinna, hearing test, Weber/Rinne.
• Otoscopic. View external canal, tympanic membrane for injection, erythemia, fluid, pus, cholesteatoma, scarring, retraction, bulging, perforation, check mobility of tympanic membrane.
• Mouth. Pharyngitis, tonsillar enlargement.
• Neck. Nuchal rigidity, lymphadenopathy, thyroid enlargement.
• Neurologic. Altered mental status, focal cranial nerves, reflexes, muscle strength, sensory findings. Ataxia, nystagmus, seizures.

- Minimal Evaluation. CBC with differential, reticulocyte count.

- Additional Testing. Throat culture for pharyngitis/tonsillitis, x-rays of mastoid for high fever, swelling or tenderness. Lumbar puncture for nuchal rigidity, altered mental status, high fever. Blood cultures with high fever. Tympanocentesis for persistent purulent effusion on appropriate antibiotics. Audiology with tympanometry for recurrent or persistent effusions or with hearing loss symptoms. Audiology exam should be a routine part of health maintenance. Annual assessment of hearing is recommended for patients on desferoxamine chelation therapy for iron overload.

Differential Diagnosis

- Otitis Externa. Inflammation, exudate in external canal only, no fever, may have pre or post auricular adenopathy.

- Acute Otitis Media. Inflammation, immobilization, retraction or bulging of tympanic membrane, purulent fluid present. Fever and leukocytosis may be present.

- Serous Otitis. Serous fluid, immobilization, no inflammation or fever are present often with atopic history.

- Bullous Myringitis. Hemorrhagic bullae on tympanic membrane seen with mycoplasma and bacterial infections.

- Mastoiditis. Past history of chronic otitis, pain, high fever, swelling behind ear or posterior canal, adenopathy. High fever, headache, stiff neck, altered mental status, seizures, focal neurologic symptoms suggest meningitis or brain abscess.

- Impaction. Cerumen, foreign body, or insect may be seen in canal.

- Temporomandibular Joint Disease. Temporomandibular tenderness, pain on opening mouth support the presence of this common entity. Mandibular bone infarctions may simulate this syndrome.

- Parotitis. Tenderness and swelling of parotid gland with inability to palpate angle of jaw indicate parotitis.

- Hearing Loss. Hearing loss is more frequent in sickle cell patients. Etiology ischemic damage to the cochlea or suckling with static blood flow in the cochlear venous system.

- Desferoxamine – is associated with sensorineural hearing losss. This is more common with high dose therapy or treatment when iron overload is not severe.

- Meniere’s Disease. Hearing loss with vertigo, nystagmus, nausea, vomiting, tinnitus and a family history support this diagnosis.

- Referred Pain. Toothache, lymphadenitis, thyroiditis can all cause pain which is perceived in the ear.

- Sensorineural hearing loss (SNHL) from sickle cell disease.

Treatment

- Otitis Externa. Cortosporin Otic Suspension iv(4) drops Q.I.D. with follow-up in one week. Exclude tympanic membrane perforation before treatment. If fungal infection or suprainfection is suspected, use Nystatin liquid as above.

- Otitis Media. With pain crisis or temperature of >38.5^oC ADMIT patient for I.V. fluids, oral antibiotics, and observation. In areas where beta lactamase producing bacteria are rare, use Amoxicillin 50 mg/kg/day in three doses P.O. q 8 hours for 10 days. Where betalactamase producing bacteria are common or with penicillin allergy use Trimethoprim 10/mg/kg/day with sulfamethoxazole in two doses P.O. q 12 hours or erythromycin 40 mg/kg/day plus sulfoxazole in four doses P.O. q 6 hours all given for 10 days. If symptoms persist for over 48 hours, re-evaluate the patient. Follow-up in two weeks. If purulent otitis persists, treat with alternative regiment or ampicillin plus clavulanate, cefaclor, or cefixine. Repeated recurrences may require tympanocentesis for culture and sensitivity testing. Acetaminophen or acetaminophen plus codeine may be needed to control pain and as antipyretics early in the treatment. Systemic decongestants, ear drops, and topical vasoconstrictors are of no proven benefit. Tympanocentesis or myringotomy may be indicated for severe pain with bulging ear drum. Serous otitis may persist in up to 40% (See below and Berman & Schmitt).

- Serous Otitis Persistent serous otitis for greater than six weeks may be treated with prednisone 1 mg/kg/day in two doses for 7 days with trimethoprim/sulfoxazole; ampicillin plus clavulanic acid, or cefaclor for four weeks. Oral decongestants and antihistamines are used, however, controlled studies have failed to document efficacy. Persistence requires audiometry, tympanometry, and consideration for PE tubes.

- Bullous Myringitis. In children, the treatment is the same as for otitis media. Consider erythromycin 500 mg P.O. q 6 hours for 10 days in adults.

- Mastoiditis. Admit STAT for I.V. antibiotics and obtain otolaryngology consult for myringotomy for culture and for possible surgery.

- Impaction. If perforation is suspected, refer to otolaryngology. Remove foreign body with irrigation if possible. Use Debrox, Cerumenex, or hydrogen peroxide for 3 - 4 days and repeat irrigation for recalcitrant cerumen.

- Temporomandibular Joint Disease. Treat with aspirin or non-steroidal anti-inflammatory and refer to oral surgery for evaluation.

- Parotitis. Mumps home with bed rest, fluids, and acetaminophen. Other types, Obtain otolaryngology consult for suspected suppurative parotitis.

- Hearing Loss. Obtain audiometry and otolaryngology evaluation.

- Meniere’s Disease. Refer for otolaryngology evaluation.

- Referred Pain. Treat underlying condition.

- Sensorineural hearing loss (SNHL). Refer for otolaryngology evaluation and possible hearing aids.

Nursing Considerations:

 Teach parents/caregivers proper administration of eardrops (straighten ear canal, then pull down and back for children, or pull up and back for adults)

 Instruct parents and caregivers to first clean the ear canal with tissue or cotton-tipped application because drainage can reduce the medication’s effectiveness

 Suggest that cotton ball may be loosely tucked into the opening of the ear canal to present the medication from leaking out

 Stress the importance of taking the medication regularly and as ordered keeping follow up appointments to avoid complications and/or hearing loss/impairment

 Emphasize the importance of hand washing before and after administration of eardrops (and in-between each ear if applicable)

 Inform parents and caregivers that hurried movements exacerbates vertigo, therefore to move slowly around patients experiencing vertigo

 Instructs parents and caregivers to report any of the following symptoms immediately: fever, irritability, lethargy and or if vomiting and earache worsens

 Pulling, tugging or scratching on the ear are early signs of ear infections in infants

 Infants should not be put to bed with a bottle

 Stress the importance of adherence to medication as prescribed to prevent hearing loss and chronic ear infections

Prevention

Rigorous follow-up with a systematic approach to diagnosis and therapy, like that outlined by Berman and Schmitt, will provide the best results and outcome. Meningitis, sepsis, and mastoiditis must always be considered when approaching a patient with otitis media. Audiometry and tympanometry provide objective measurements to follow the patient with persistent or recurrent otitis. Exposure to loud noise should be avoided or ear protection should be provided. All patient should be screened by audiology, especially children every 2 years. All patients on deferoxamine should have annual hearing screening.

Patient and Parent Education

Parents need reassurance that hearing will not be impaired if medications are taken as prescribed and follow-up appointments are kept. They should be instructed to return for re-evaluation if earache, fever, irritability, lethargy, or vomiting get worse or persist for more than 48 hours. Education about the importance of taking medications regularly must be reinforced, especially when prolonged treatment is required for persistent or recurrent problems. Parents should be alert for signs of hearing loss.

References

MacDonald CB, Bauer PW, Cox LC, McMahon L Otologic findings in a pediatric cohort with sickle cell disease. Int J Pediatr Otorhinolaryngol 1999 Jan 25;47(1):23-8

Gentry B, Davis P, Dancer J Failure rates of young patients with sickle cell disease on a hearing screening test. Percept Mot Skills 1997 Apr;84(2):434

Chiodo AA, Alberti PW, Sher GD, Francombe WH, Tyler B Desferrioxamine ototoxicity in an adult transfusion-dependent population. J Otolaryngol 1997 Apr;26(2):116-22

Giebink GS. The microbiology of otitis media. Pediatr Infect Dis J 8:S18, 1989.

Baker RC. Acute Otitis Media in Handbook of Pediatric Primary Care. Baker RC Editor. Lippencott Williams and Wilkins 1999.

Berman S & Schmitt BD. Ear, Nose, and Throat. In Current Pediatric Diagnosis & Treatment. Hathaway WE, Groothuis JR, Hay WW, Paisley JW eds. Appleton & Lange. Norwalk Conn. 1991.

Teele DE, Pelton DW, Klein JO. Bacteriology of acute otitis media unresponsive to initial antibiotic therapy. J. Pediatr. 98:537, 1981.

Bluestone CD. Management of otitis media in infants and children. Pediatr. J. Infect. Dis. 7:512, 1988.

Healy GB, Smith RH. Current concepts in the management of otitis media with effusion. Amer. J. Otolaryngol. 2:138, 1981.

Roberts DB. The etiology of bullous myringitis and the role of mycoplasma in ear disease. a review. Pediatrics 65:761, 1980.

Mandel EM et al. Efficacy of amoxicillin with and without decongestant antihistamine for otitis media with effusion in children. New Engl. J. Med. 316:432, 1987.

Friedman MJ, Here GR, Luban NLC, Williams I. Sickle cell anemia and hearing. Ann. Otol. Rhinol. Laryngol. 89:342, 1980.

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Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000