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There Is Something in the Ear: Mondini Dysplasia
There Is Something in the Ear: Mondini Dysplasia
A
3-year-old boy who was immunized against pneumococcus with a 7-valent conjugate vaccine presented to our hospital with fever, headache, and vomiting for 1 day. Pneumococcal meningitis was diagnosed, and he had an uneventful recovery after complete antimicrobial treatment. Two months later, recurrent pneumococcal meningitis occurred, and cranial computed tomography was performed. Bilateral 3 semicircular canals were revealed, and accumulation of fluid in the left middle ear and mastoid area was shown (Figure 1). There was incomplete partition of the cochlea and dilatation of the vestibule, aqueduct, and ampulla of the left inner ear (Figure 2 and Video; available at www.jpeds.com). The diagnosis of Mondini dysplasia was made, and the cisternography and immune studies were unremarkable. Surgical repair was performed, and there was no recurrence after 12 months of follow-up. Mondini dysplasia is an inner ear malformation thought to be caused by the arrest of neural tube development during the seventh week of gestation. It is characterized by cochlear malformation with the dilatation of the vestibule, aqueduct, and ampulla, as well as the incomplete partitioning of the co-
chlea.1 In this condition, there are less than the expected number of turns (2.5) in the cochlea, and the cochlea is confluent and sac-like because of the absence of interscalar septa, as observed in our patient.1 A perilymph fistula, resulting in an abnormal communication between the subarachnoid space and the middle ear, is common in patients with Mondini dysplasia; therefore, recurrent, retrograde meningitis is a common complication.2,3 Although surgical repair of anatomic defects could prevent the occurrence of meningitis, the clinical manifestations of Mondini dysplasia usually remain obscure, and the condition remains undiagnosed until recurrent meningitis occurs. This may lead to permanent neurologic damage and sequelae.4 Cranial high-resolution computed tomography is the preferred diagnostic approach for Mondini dysplasia.5 Furthermore, vigilance and familiarity with distinctive radiologic presentations can contribute to a timely diagnosis. In patients with recurrent meningitis, high suspicion for Mondini dysplasia should be maintained. Moreover, careful history taking and image reading may provide a clue for accurate diagnosis and timely treatment. n
Chi-Hone Lien, MD Hung-Yang Chang, MD Yu-Peng Liu, MD Hsin Chi, MD Nan-Chang Chiu, MD Chien-Yu Lin, MD Department of Pediatrics and Radiology Hsinchu Mackay Memorial Hospital Hsinchu City, Taiwan Department of Pediatrics Mackay Memorial Hospital Taipei, Taiwan
References
Figure 1. Temporal bone multislice computed tomography revealed accumulation of fluid in the left middle ear and mastoid area (black arrows) and the typical 3 semicircular canals in bilateral inner ears (white arrows).
J Pediatr 2014;165:638. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.04.048
638
1. Zheng Y, Schachern PA, Cureoglu S, Mutlu C, Dijalilian H, Paparella MM. The shortened cochlea: its classification and histopathologic features. Int J Pediatr Otorhinolaryngol 2002;63:29-39. 2. Lin CY, Lin HC, Peng CC, Lee KS, Chiu NC. Mondini dysplasia presenting as otorrhea without meningitis. Pediatr Neonatol 2012;53:371-3. 3. Syal R, Tyagi I, Goyal A. Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias. Int J Pediatr Otorhinolaryngol 2005;69:983-8. 4. Wiener-Vacher SR, Obeid R, Abou-Elew M. Vestibular impairment after bacterial meningitis delays infant posturomotor development. J Pediatr 2012;161:246-51.e1. 5. Mantur M, Lukaszewicz-Zajac M, Mroczko B, Kulakowska A, Ganslandt O, Kemona H, et al. Cerebrospinal fluid leakage–reliable diagnostic methods. Clin Chim Acta 2011;412:837-40.
Vol. 165, No. 3 September 2014
Figure 2. Compared with the normal right inner ear, incomplete partition of the cochlea and dilatation of the vestibule, aqueduct, and ampulla of the left inner ear were revealed (white arrows). The serial images were converted to the Video for better understanding.
638.e1
A
3-year-old boy who was immunized against pneumococcus with a 7-valent conjugate vaccine presented to our hospital with fever, headache, and vomiting for 1 day. Pneumococcal meningitis was diagnosed, and he had an uneventful recovery after complete antimicrobial treatment. Two months later, recurrent pneumococcal meningitis occurred, and cranial computed tomography was performed. Bilateral 3 semicircular canals were revealed, and accumulation of fluid in the left middle ear and mastoid area was shown (Figure 1). There was incomplete partition of the cochlea and dilatation of the vestibule, aqueduct, and ampulla of the left inner ear (Figure 2 and Video; available at www.jpeds.com). The diagnosis of Mondini dysplasia was made, and the cisternography and immune studies were unremarkable. Surgical repair was performed, and there was no recurrence after 12 months of follow-up. Mondini dysplasia is an inner ear malformation thought to be caused by the arrest of neural tube development during the seventh week of gestation. It is characterized by cochlear malformation with the dilatation of the vestibule, aqueduct, and ampulla, as well as the incomplete partitioning of the co-
chlea.1 In this condition, there are less than the expected number of turns (2.5) in the cochlea, and the cochlea is confluent and sac-like because of the absence of interscalar septa, as observed in our patient.1 A perilymph fistula, resulting in an abnormal communication between the subarachnoid space and the middle ear, is common in patients with Mondini dysplasia; therefore, recurrent, retrograde meningitis is a common complication.2,3 Although surgical repair of anatomic defects could prevent the occurrence of meningitis, the clinical manifestations of Mondini dysplasia usually remain obscure, and the condition remains undiagnosed until recurrent meningitis occurs. This may lead to permanent neurologic damage and sequelae.4 Cranial high-resolution computed tomography is the preferred diagnostic approach for Mondini dysplasia.5 Furthermore, vigilance and familiarity with distinctive radiologic presentations can contribute to a timely diagnosis. In patients with recurrent meningitis, high suspicion for Mondini dysplasia should be maintained. Moreover, careful history taking and image reading may provide a clue for accurate diagnosis and timely treatment. n
Chi-Hone Lien, MD Hung-Yang Chang, MD Yu-Peng Liu, MD Hsin Chi, MD Nan-Chang Chiu, MD Chien-Yu Lin, MD Department of Pediatrics and Radiology Hsinchu Mackay Memorial Hospital Hsinchu City, Taiwan Department of Pediatrics Mackay Memorial Hospital Taipei, Taiwan
References
Figure 1. Temporal bone multislice computed tomography revealed accumulation of fluid in the left middle ear and mastoid area (black arrows) and the typical 3 semicircular canals in bilateral inner ears (white arrows).
J Pediatr 2014;165:638. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.04.048
638
1. Zheng Y, Schachern PA, Cureoglu S, Mutlu C, Dijalilian H, Paparella MM. The shortened cochlea: its classification and histopathologic features. Int J Pediatr Otorhinolaryngol 2002;63:29-39. 2. Lin CY, Lin HC, Peng CC, Lee KS, Chiu NC. Mondini dysplasia presenting as otorrhea without meningitis. Pediatr Neonatol 2012;53:371-3. 3. Syal R, Tyagi I, Goyal A. Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias. Int J Pediatr Otorhinolaryngol 2005;69:983-8. 4. Wiener-Vacher SR, Obeid R, Abou-Elew M. Vestibular impairment after bacterial meningitis delays infant posturomotor development. J Pediatr 2012;161:246-51.e1. 5. Mantur M, Lukaszewicz-Zajac M, Mroczko B, Kulakowska A, Ganslandt O, Kemona H, et al. Cerebrospinal fluid leakage–reliable diagnostic methods. Clin Chim Acta 2011;412:837-40.
Vol. 165, No. 3 September 2014
Figure 2. Compared with the normal right inner ear, incomplete partition of the cochlea and dilatation of the vestibule, aqueduct, and ampulla of the left inner ear were revealed (white arrows). The serial images were converted to the Video for better understanding.
638.e1